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About half of the blood cancers that occur each year are lymphomas, or cancers of the lymphatic system. This system - composed of lymph nodes in your neck, armpits, groin, chest, and abdomen - removes excess fluids from your body and produces immune cells. Abnormal lymphocytes, a type of white blood cell that fights infection, become lymphoma cells, which multiply and collect in your lymph nodes. Over time, these cancerous cells impair your immune system.
Lymphomas are divided into two categories: Hodgkin lymphoma and non-Hodgkin lymphoma. About 12 percent of people with lymphoma have Hodgkin lymphoma. Because of breakthrough research , this once fatal diagnosis has been transformed into a curable condition. Most non-Hodgkin lymphomas are B-cell lymphomas, and either grow quickly (high-grade) or slowly (low-grade). There are over a dozen types of B-cell non-Hodgkin lymphomas. The rest are T-cell lymphomas, named after a different cancerous white blood cell, or lymphocyte.
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How Lymphoma Develops
Many lymphoma patients are able to lead active lives as they receive treatment for their symptoms and are monitored by their doctors.
Am I at Risk?
The exact causes of lymphoma remain unknown; however, the following factors increase your risk of developing the disease:
- Having an autoimmune disease
- Diet high in meats and fat
- Being exposed to certain pesticides
Symptoms of lymphoma include the following:
- Swollen lymph nodes in the neck, armpits, or groin
- Weakness and fatigue
- Weight loss
- Difficulty breathing or chest pain
How is Lymphoma Treated?
Your doctor will perform a lymph node biopsy to diagnose lymphoma. Additional tests are then conducted to determine the stage (extent) of the lymphoma including blood tests, bone marrow biopsies, and imaging tests, such as a CT scan or PET scan. Imaging tests show whether the lymphoma has spread to other parts of your body, like the spleen and lungs. Decisions about treatment are then determined by your doctor, who will consider your age, general health, and stage and type of lymphoma. Hodgkin lymphoma is one of the most curable types of cancer.
Treatment options include the following:
- Chemotherapy
- Chemotherapy and radiation that directly targets the lymphoma
- Biological therapies, such as antibodies, directed at lymphoma cells
- Stem cell transplant
For some patients, participating in a clinical trial provides access to experimental therapies. If you are diagnosed with lymphoma, talk with your doctor about whether joining a clinical trial is right for you.
Is Lymphoma Preventable?
Because the cause of lymphoma remains unknown, there is no real way to prevent it. However, if you think you may be exhibiting signs of lymphoma, being aware of the risk factors and symptoms and talking with your doctor are critical to early diagnosis and treatment. It is especially important if you have a family history of lymphoma to look out for symptoms and share your family medical history with your doctor.
If you suspect that you have or are at risk for lymphoma, talk with your doctor about detection and treatment. Depending on your physical condition, genetics, and medical history, you may be referred to a hematologist, a doctor who specializes in blood conditions.
Lymphoma: A Patient's Journey
Where Can I Find More Information?
If you find that you are interested in learning more about blood diseases and disorders, here are a few other resources that may be of some help:
Results of Clinical Studies Published in Blood
Search Blood , the official journal of ASH, for the results of the latest blood research. While recent articles generally require a subscriber login, patients interested in viewing an access-controlled article in Blood may obtain a copy by e-mailing a request to the Blood Publishing Office .
Patient Groups
A list of Web links to patient groups and other organizations that provide information.
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Hodgkin Lymphoma
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Introduction
- Hodgkin lymphoma (HL) is a haematological malignancy that arises from B lymphocytes in the lymphatic system.
In the United Kingdom, 2,100 people are diagnosed each year, with a peak incidence in young adults aged 20 – 34 years and in older adults aged over 70 years.
Overall, the prognosis is good with 75% of patients with HL surviving for ten years or more.
Prognosis is better in the younger population than in the older population. Younger patients under the age of 40-years-old have a five-year survival rate of 95% whereas older patients over the age of 70-years-old have a five-year survival rate of less than 50%. 1,2
The lymphatic system is a network of tissues and organs that play a key role in the immune system. This network consists of lymph nodes, lymphatic vessels, lymphatic organs and lymphatic fluid. 3
![typical presentation of lymphoma typical presentation of lymphoma](https://geekymedics.com/wp-content/uploads/2021/01/Figure-1-The-Lymphatic-System-600x686.png)
Lymphatic fluid contains a high number of lymphocytes and it is the mutation of these cells inside lymphoid tissues that results in a lymphoma.
Hodgkin lymphoma occurs when B lymphocytes, derived from the germinal centres of lymphoid tissues, mutate and lead to the presence of large , multi-nucleated giant cells called ‘Reed-Sternberg’ cells and large , mono-nucleated cells called malignant ‘Hodgkin cells’. 4,5
Classification
There are two main types of Hodgkin lymphoma (HL), classical Hodgkin lymphoma (which accounts for 95% of HL cases) and nodular lymphocyte-predominant Hodgkin lymphoma (which accounts for 5% of HL cases).
Classical Hodgkin lymphoma (cHL) is further subclassified into four types.
Table 1 . Classical Hodgkin lymphoma sub-classification.
|
|
| 70% of cHL cases. Mediastinal mass is common.
|
| 25% of cHL cases. Prevalent in patients with HIV and in developing countries. Splenic infiltration is seen in 30% of patients. |
| 5% of cHL cases. Mediastinal mass is rare.
|
| <1% of cHL cases. Prevalent in patients with HIV and in developing countries. |
Nodular lymphocyte-predominant Hodgkin lymphoma is a more indolent disease with little in common with cHL. It is associated with a risk of transformation to a high grade (rapidly growing) non-Hodgkin lymphoma. 7
Risk factors
The following risk factors are associated with an increased likelihood of developing HL:
- Epstein-Barr virus (EBV): it is estimated that around 40% of Hodgkin lymphoma cases in the UK are related to EBV infection. However, most people who have glandular fever will not develop cancer as a result. 3
- Human immunodeficiency virus (HIV): it is estimated that the risk of developing Hodgkin lymphoma is 11 times higher than that of the general population
- Immunosuppression: immunosuppressant drugs and certain autoimmune conditions such as rheumatoid arthritis increase the risk of developing HL
- Previous history of non-Hodgkin lymphoma (NHL)
- First-degree relative family history of Hodgkin lymphoma, non-Hodgkin lymphoma (NHL) or chronic lymphocytic leukaemia (CLL)
- Cigarette smoking
Clinical features
The most common symptom of Hodgkin lymphoma (HL) is a painless , rubbery , enlarged lymph node/nodes, typically in the cervical or supraclavicular region. 7
Other typical symptoms of Hodgkin lymphoma include:
- B symptoms: fever >38°C, drenching night sweats and unintentional weight loss of >10% within the last 6 months. These symptoms affect 25% of patient with HL.
- Chest discomfort +/- cough or dyspnoea: a mediastinal mass is present in 80% of patients with HL and may cause these symptoms 8
- Abdominal discomfort or pain: if abdominal lymphatic organs such as the liver or spleen have been affected
- Alcohol-induced pain at nodal sites: this is a ‘classical’ textbook symptom of Hodgkin Lymphoma but not often seen
Clinical examination
All patients with suspected Hodkin lymphoma require a through lymphoreticular system examination .
On examination, clinical findings in HL may include:
- Lymphadenopathy
- Hepatomegaly
- Splenomegaly
- Superior vena cava (SVC) syndrome: a mediastinal mass may cause SVC obstruction
- Paraneoplastic syndromes such as cerebellar degeneration, neuropathy or Guillain-Barré syndrome
Differential diagnoses
The clinical presentation of Hodgkin lymphoma is similar to several other conditions including:
- Infectious mononucleosis
- Non-Hodgkin lymphoma
- Acquired immunodeficiency syndrome (AIDS)
- Tuberculosis
- Sarcoidosis
- Toxoplasmosis
Investigations
Laboratory investigations.
Relevant laboratory investigations include:
- FBC: to investigate for leukaemia, infectious mononucleosis and other causes of lymphadenopathy
- U&Es: to provide a baseline measurement before treatment
- LFTs: reduced albumin levels are associated with a poorer prognosis
- LDH: increased levels are associated with a poorer prognosis
- ESR: increased levels are associated with a poorer prognosis
- Tests to exclude differential diagnoses: for example, Monospot® test for infectious mononucleosis, sputum culture for TB, viral screen including HIV, hepatitis B virus (HBV) and hepatitis C virus (HCV) tests
Relevant imaging investigations include:
- Chest X-ray: to assess for intrathoracic lymphadenopathy and mediastinal expansion
- Contrast-enhanced CT neck, chest, abdomen and pelvis: usually performed when a patient first presents and is sometimes used for staging.
- Positron emission tomography CT (PET-CT): now the gold-standard for staging in cHL , and is repeated during treatment to allow guided therapy according to response (aiming to minimise toxicity by reducing chemotherapy intensity when possible)
Specialist investigations
Lymph node excision biopsy is required for diagnosis and classification of HL type.
On light microscopy, the hallmark cell is the Reed-Sternberg cell which is a giant malignant multi-nucleated cell that is often referred to as being “owl-like”. A collection of non-malignant immune cells also surround the Reed-Sternberg cells.
![typical presentation of lymphoma A Reed-Sternberg cell, a hallmark of Hodgkin lymphoma](https://geekymedics.com/wp-content/uploads/2021/01/Figure-2-Reed-Sternberg-Cell-600x384.jpg)
Hodgkin cells are giant malignant mono-nucleated cells and tend to be present surrounding Reed-Sternberg cells. 5
Using immunocytochemistry , CD15 and CD30 antigens are positively expressed on Reed-Sternberg cells.
A bone marrow biopsy is less frequently used as PET/CT can detect marrow involvement. 7
Once Hodgkin lymphoma is diagnosed, the disease is staged to determine prognosis and guide treatment options. Staging is performing using the Ann Arbor staging system . 8
The following table is a summarised version of the staging criteria; any more detail is beyond the scope of undergraduate learning.
Table 2 . A summarised version of the Ann Arbor staging system.
| |
| Involvement of one lymph-node region or lymphoid structure (e.g. spleen or thymus). |
| Two or more lymph node regions on the same side of the diaphragm. |
| Lymph nodes on both sides of the diaphragm. |
| Involvement of extranodal site(s) beyond that designated E (see below). |
| |
| No symptoms |
| Fever >38°C, drenching night sweats, weight loss of more than 10% over 6 months |
Prior to treatment, patients usually undergo cardiac function testing, pulmonary function testing and reproductive counselling due to the potential side effects of chemotherapy and radiotherapy.
Due to the increased risk of opportunistic infections following chemotherapy, patients are also usually vaccinated with the following: 8
- Polyvalent pneumococcal vaccine
- Influenza vaccine
- Meningococcal group C conjugate vaccine
- Haemophilus influenzae type b vaccine
Initial therapy 8
Early-stage disease (stage IA, IB, IIA) is usually treated with one or more cycles of combination chemotherapy plus radiotherapy .
Advanced stage (stage IIB or above) is usually treated with a more intensive chemotherapy course; often without radiotherapy unless a particularly large mass is present.
The most commonly used chemotherapy combination regimes in Hodgkin lymphoma are:
- ABVD: Doxorubicin (used to be called A driamycin®), B leomycin, V inblastine and D acarbazine
- BEACOPP: B leomycin, E toposide, Doxorubicin ( A driamycin®), C yclophosphamide, Vincristine ( O ncovin®), P rocarbazine, P rednisolone
Relapsed disease 10
Regardless of stage, relapsed disease is usually treated with high dose chemotherapy (HDCT) followed by autologous stem cell transplant (ASCT).
HDCT aims to eradicate all HL cancer cells , however, bone marrow stem cells are also destroyed during the process.
The re-transfusion of the patient’s own haematopoietic stem cells (salvaged prior to HDCT) aims to promote a quicker recovery of bone marrow function and reduce the duration period of profound immunosuppression in the patient.
The chemotherapeutic regime chosen for patients eligible for ASCT is based on individual patient factors.
If a patient cannot tolerate intensive HDCT and ASCT, then combination chemotherapy and radiotherapy is considered. A more intensive chemotherapy regimen than that used in the initial therapeutic plan is usually offered.
If a patient cannot tolerate the toxicities associated with more intensive regimens, palliative chemotherapy and/or radiotherapy is considered.
Blood transfusion
If a transfusion of blood products is required, patients with or treated for Hodgkin lymphoma (at any stage of the disease) must only receive irradiated blood products . This is a lifelong requirement .
Irradiated blood products are used to reduce the risk of transfusion-associated graft-versus-host disease .
Complications
Disease-related complications.
Hodgkin lymphoma causes immunosuppression . The clonal expansion of B lymphocytes are abnormal and do not function properly. Patients are at a particularly higher risk of infection if there is bone marrow involvement.
Treatment-related complications
Treatment-related complications may include:
- Neutropenia: due to the effect of the chemotherapy on the bone marrow. Antibiotics are urgently required if patients are suspected to be neutropenic and have symptoms of infection or pyrexia ( neutropenic sepsis ). Patients can also be given granulocyte colony-stimulating factor (G-CSF) to stimulate the production of neutrophils which may reduce the duration of chemotherapy-induced neutropenia and therefore reduce the incidence of associated sepsis.
- Secondary solid tumours: particularly in the lung, skin, breast and gastrointestinal tract 1
- Secondary leukaemias: especially acute myeloid leukaemia
- Subfertility: patients should be counselled prior to treatment
- Cardiovascular disease: secondary to adriamycin/doxorubicin
- Lung fibrosis: secondary to bleomycin and presents months to years after treatment
- Endocrine dysfunction
- Nausea and vomiting
- Hodgkin lymphoma is characterised by the presence of Reed-Sternberg cells which are derived from germinal centre B lymphocytes.
- The main risk factor for Hodgkin lymphoma is previous EBV infection.
- Hodgkin lymphoma classically presents with a painless , rubbery , enlarged lymph node +/- ’ B symptoms ’
- The diagnosis is made by lymph node biopsy. PET-CT is used for staging in cHL.
- Management of Hodgkin lymphoma is with combination chemotherapy (usually either ABVD or BEACOPP) plus radiotherapy in early-stage disease or a longer, more intensive course of chemotherapy in advanced-stage disease. High dose chemotherapy and autologous stem cell transplantation are considered in relapsed disease.
- Complications include immunosuppression, low blood cell counts, secondary cancers, lung fibrosis, cardiovascular disease and subfertility.
- Prognosis is good with a 75% ten-year survival rate.
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References .
- World Health Organisation. Hodgkin Lymphoma (Adult). Published in 2014. Available from: [ LINK ]
- Cancer Research UK. Hodgkin lymphoma Incidence Statistics. Published in 2015. Available from: [ LINK ]
- Cancer Research UK. About Hodgkin lymphoma. Published in 2020. Available from: [ LINK ]
- Patient UK. Hodgkin’s Lymphoma . Published in 2019. Available from: [ LINK ]
- Küppers R et al. Identification of Hodgkin and Reed-Sternberg cell-specific genes by gene expression profiling. Journal of Clinical Investigation. Published in 2003. Available from: [ LINK ]
- Cancer Research UK. Diagram of the Lymphatic system. License: [Public Domain]. Available from: [ LINK ]
- Townsend W et al. Hodgkin’s Lymphoma in Adults. The Lancet. Published in 2012. Available from: [ LINK ]
- Follows G et al. Guidelines for the first-line management of classical Hodgkin lymphoma. British Journal of Haematology. Published in 2014. Available from: [ LINK ]
- National Cancer Institute. Image of Reed-Sternberg Cell. License: [Public Domain]. Available from: [ LINK ]
- Ansell SM et al. Hodgkin lymphoma: 2012 update on diagnosis, risk-stratification, and management. American Journal of Hematology. Published in 2012. Available from: [ LINK ]
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The Clinical Presentation of Lymphomas
![typical presentation of lymphoma typical presentation of lymphoma](https://ohcare.com/wp-content/uploads/2015/04/Hodgkin-Lymphoma-Nodular-Lymphocyte-Predominant-OHC.jpg)
From Miguel Islas-Ohlmayer, M.D.
April 20, 2015
![typical presentation of lymphoma Islas-Ohlmayer Miguel MD - PHOTO](https://ohcare.com/wp-content/uploads/2013/05/Islas-Ohlmayer-Miguel-MD-PHOTO-200x300.jpg)
Miguel Islas-Ohlmayer, M.D.
Lymphomas, blood cell tumors that usually originate in lymphatic tissues (and can spread to other organs), are among the most diverse and most curable of all malignancies. There are two major groups of lymphomas: About 90 percent are non-Hodgkin lymphomas (NHLs) and about 10 percent are Hodgkin lymphomas (HLs).
NON-HODGKIN LYMPHOMAS (NHLs) Let’s start with NHLs, malignant neoplasms derived from B cell progenitors, T cell progenitors, mature B cells, mature T cells, or (rarely) natural killer cells. NHLs are the seventh most common type of cancer in American men and the sixth most common in American women. The American Cancer Society estimates nearly 72,000 cases and 20,000 deaths in 2015.
Clinical Presentation
Most NHL patients are first seen by their PCPs because they are showing signs of lymphadenopathy, although the enlarged nodes are usually painless. Here’s what your patients might present with:
- Aggressive NHLs usually present acutely or subacutely with a rapidly growing mass, systemic B symptoms (fever, night sweats, weight loss), and/or elevated levels of serum lactate. Treatment in these cases should be treated promptly to manage symptoms, slow or revert progression, and optimize survival.
- Indolent lymphomas often present only with slow-growing lymphadenopathy, hepatomegaly, splenomegaly, or cytopenias.
- Less commons presentations include skin rash, pruritus, hypersensitivity to insect stings and bites, generalized fatigue, malaise, fever of unknown origins, ascites, and effusions.
- Patients with primary GI track lymphoma may present with anorexia, weight loss, nausea and vomiting, chronic pain, abdominal fullness, early satiety, symptoms of visceral obstruction, or even acute perforation and GI hemorrhage.
- Patients with primary CNS lymphoma may have headache, lethargy, facial neurologic symptoms, seizures, paralysis, spinal cord compression or lymphomatous meningitis.
Risk Factors:
- Getting older is a strong risk factor for lymphoma overall, with most cases occurring in people in their 60s or older
- The risk of NHL is higher in men than in women, but there are certain types of non-Hodgkin lymphoma that are more common in women
- NHL in the United States is more common in Caucasians than African Americans or Asians
- Inordinate contact with chemicals such as benzene, and certain herbicides and insecticides
- Inordinate radiation exposure
- History of autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, Sjogren disease, and celiac sprue disease
- History of infections that directly transform lymphocytes such as T-cell leukemia/lymphoma virus (HTLV-1) and the Epstein-Barr virus (EBV)
- History of therapy with immunosuppressant drugs used in organ transplants, autoimmune disease, and HIV
- Inherited immune disorders such as hypogammaglobulinemia and Wiskott-Aldrich syndrome
HODGKIN LYMPHOMAS (HLs)
There are two major types of Hodgkin lymphomas: classical Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma. Both are marked by the presence of HLs of the Reed-Sternberg cell. Note that this type of cell also can be found in reactive lymphadenopathy (such as infectious mononucleosis) and more rarely in other types of non-Hodgkin lymphomas. Both are marked by the presence of Reed-Sternberg cells.
According to the National Cancer Institute, the five-year survival rate (2004-2010) is 85.3 percent. And the number of deaths has been declining. Clinical Presentation:
Because there are no widely recommended screening tests for Hodgkin lymphomas, you will need to pay attention to your patient’s risk factors in combination with these clinical presentations:
- Asymptomatic lymphadenopathy, above the diaphragm 80% of time but also found under the arm or in the groin
- Intermittent fever is observed in approximately 35% of cases; infrequently, the classic Pel-Ebstein fever is observed (high fever for 1-2 weeks, followed by an afebrile period of 1-2 weeks)
- Chest pain, cough, shortness of breath, or a combination of those may be present due to a large mediastinal mass or lung involvement
- Patients may present with pruritus
- Pain at sites of nodal disease, precipitated by drinking alcohol, occurs in fewer than 10% of patients but is specific for Hodgkin lymphoma
- Back or bone pain may rarely occur
- A family history is also helpful; in particular, nodular sclerosis Hodgkin lymphoma (NSHL) has a strong genetic component and has often previously been diagnosed in the family
- History of infectious mononucleosis caused by Epstein Barr virus (EBV)
- Occurs slightly more often in men than women
- Most common with young adults (peaking at about 20) and older adults (peaking at about 65); and those in the United States, Canada, and Northern Europe
- History of younger siblings with the disease (but only by 5% per the American Cancer Society), and especially so with identical twins
- People from higher socioeconomic backgrounds (though the correlation is still unclear)
- People infected with HIV
- Lymphoma is widely diverse group of diseases that originate in lymphatic tissues
- Because the symptoms caused by lymphomas are often the same as those from infections, patient history, risk factors, and clinical presentation need to be viewed in total
- Presentation most often includes asymptomatic lymphadenopathy, usually found in the neck (above the diaphragm)
- Precise diagnosis and classification requires evaluation of lymph node tissue obtained via adequately sized biopsy
- Hodgkin lymphomas (HLs) and non-Hodgkin lymphomas (NHLs) are curable in the majority of patients
Miguel Islas-Ohlmayer, M.D. , is a hematologist and blood and marrow transplant physician. Board certified in internal medicine and hematology, his main interests include hematologic malignancies as well as blood and marrow stem cell transplantation. Dr. Islas-Ohlmayer practices at our OHC Kenwood office and is a clinician at the Blood Cancer Center at The Jewish Hospital-Mercy Health. The Blood Cancer Center, powered by OHC physicians, is a recent recipient of The Joint Commission’s Gold Seal of Approval for Blood & Marrow Transplants .
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Lymphomas: pathogenesis, clinical features and diagnosis
Lymphomas are some of the most common cancers in the UK, with a wide variation in disease progression and prognosis between subtypes.
![typical presentation of lymphoma Coloured micrograph of lymphoma cancer cells in a lymph node](https://pharmaceutical-journal.com/wp-content/uploads/2021/01/lymphoma-cancer-cells-in-lymph-node-micrograph-14-927x617.jpg)
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The lymphomas are a large group of blood cancers with many subtypes. Hodgkin lymphoma has an incidence of 2.8 per 100,000 people per year in the UK, while non-Hodgkin lymphoma has an incidence of 15.5 per 100,000 people per year.
Both forms typically present with painless enlarged lymph nodes. Around a quarter of patients with Hodgkin lymphoma will also have night sweats, unexplained fever and weight loss. Diagnosis is made following a lymph node biopsy, a CT scan, and – in non-Hodgkin lymphoma – a bone marrow biopsy.
The lymphomas are a heterogenous group of blood cancers caused by the clonal proliferation of B or T lymphocytes. There are a large number of recognised subtypes of lymphoma, and it is beyond the scope of these articles to discuss each of them individually. Instead, the main focus will be on Hodgkin lymphoma and the most common forms of non-Hodgkin lymphoma.
Incidence and risk factors
Hodgkin lymphoma has an incidence in the UK of 2.8 per 100,000 people per year, which translates into just over 1,800 new cases per year [1] . The incidence of both Hodgkin lymphoma and non-Hodgkin lymphoma has increased by 11-15% in the past decade. This is most likely because of a combination of better diagnosis and reporting, the ageing population and an increase in the number of patients with a compromised immune system, such as those with HIV and AIDS.
Hodgkin lymphoma has a bimodal distribution, with an initial peak in young adults aged 20–24 years and a second peak between the ages of 70 years and 80 years, although it can occur at any age [1] .
The disease is slightly more common in men, with an incidence ratio of 1.2:1. The cause of Hodgkin lymphoma is not known, but it does have a strong association with being infected with Epstein-Barr virus, which is implicated in 45% of cases. It also occurs more commonly in patients who are immunocompromised; HIV infection is associated with an 11-fold increase in risk of Hodgkin lymphoma [1] , and patients who are receiving immunosuppressant therapy following an organ transplant or with autoimmune conditions such as rheumatoid arthritis and systemic lupus erythematosus are also at increased risk.
A small increase in risk of Hodgkin lymphoma has also been associated with tobacco exposure, having a first degree relative with the disease, and obesity. Rates of Hodgkin lymphoma in younger patients are lower for those with three or more siblings, suggesting that exposure to common childhood infections may somehow reduce the risk of developing the disease [2] .
Non-Hodgkin lymphoma has an incidence in the UK of 15.5 per 100,000 people per year, with almost 14,000 new cases reported in 2011 [1] . It is the sixth most common type of cancer in the UK, and accounts for about 4% of all cancers. It has a relatively good prognosis and, despite its high incidence, is the tenth most common cause of cancer death in the UK.
The incidence of non-Hodgkin lymphoma correlates closely with increasing age, and the majority of cases occur in patients aged 65 years or older. One exception to this rule is the relatively uncommon Burkitt’s lymphoma, in which almost 50% of cases occur in patients younger than 45 years.
There is a strong association between immunodeficiency, such as HIV infection, and risk of developing non-Hodgkin lymphoma. Recipients of organ transplantation who are receiving immunosuppressants such as ciclosporin or tacrolimus are at risk of developing post-transplant lymphoproliferative disease (PTLD), a proliferation of B cells caused by the Epstein-Barr virus that, if untreated, can progress to non-Hodgkin lymphoma.
Burkitt’s lymphoma is a highly aggressive form of non-Hodgkin lymphoma that, in its endemic form, is associated with malarial regions of equatorial Africa. In the UK, it accounts for about 2% of cases of lymphoma. It is more common in children and young adults.
The Epstein-Barr virus is implicated in the development of Burkitt’s lymphoma, although its overall importance as a risk factor is much less than for Hodgkin lymphoma.
Helicobacter pylori infection is strongly associated with mucosa-associated lymphoid tissue (MALT) lymphoma, a form of non-Hodgkin lymphoma that occurs in the stomach. H.pylori eradication regimens are the mainstay of treatment for this relatively rare subtype.
Other risk factors for non-Hodgkin lymphoma include hepatitis B and hepatitis C, working in rubber production and exposure to chemicals such as benzene and ethylene oxide. There is no proven association between smoking and an increased risk of non-Hodgkin lymphoma.
Classification
A variety of classification systems has been developed for lymphomas. The World Health Organization (WHO) classification, last updated in 2008, is currently the most widely used and recognises more than 50 different subtypes [3] .
Hodgkin lymphoma can itself be subdivided into two forms: classic Hodgkin lymphoma, which accounts for 95% of cases, and nodular lymphocyte predominant Hodgkin lymphoma.
The simplest way of classifying non-Hodgkin lymphomas is by the cell of origin. More than 90% originate in B-lymphocytes, with less than 10% being T-cell or NK cell lymphomas.
Clinically, it is often useful to separate non-Hodgkin lymphoma into aggressive (high grade) and indolent (low grade) forms (see ‘Aggressive and indolent lymphomas’).
Example | Diffuse large B cell lymphomaBurkitt’s lymphoma | Follicular lymphomaMarginal zone lymphoma |
Presentation | Rapid onset of symptoms | Insidious onset |
Natural history | Rapidly fatal if untreated | Slow growing and treatment not always required at time of diagnosis |
Sensitivity to chemotherapy | Potentially curable | Responses seen with chemotherapy but rarely curable |
Presentation
Hodgkin lymphoma commonly presents with painless swollen lymph nodes (lymphadenopathy), often affecting the cervical or supraclavicular nodes in the neck. About 25% of patients present with the three ‘B symptoms’: night sweats, unexplained fever and weight loss of more than 10% over six months. These symptoms are associated with a poorer prognosis [4] . Other presenting features include fatigue, itching and alcohol-induced pain.
Non-Hodgkin lymphoma also classically presents with painless enlarged lymph nodes. These are usually widespread in indolent lymphomas (such as follicular lymphoma), whereas progression is more rapid and often accompanied by B symptoms in aggressive lymphomas, such as diffuse large B-cell lymphoma.
In both Hodgkin lymphoma and non-Hodgkin lymphoma, patients are more likely to be unwell due to chemotherapy side effects than their cancer. A minority of patients will have lymphoma present in the bone marrow, which can lead to symptoms related to myelosuppression. These include fatigue, breathlessness, increased susceptibility to infections and unexpected bruising or bleeding.
Rarely, the location of the lymphoma mass may cause life-threatening complications such as spinal cord compression or obstruction of the superior vena cava. These are medical emergencies that require urgent treatment with chemotherapy or radiotherapy.
A lymph node or extranodal tissue biopsy is used to diagnose lymphoma, and immunohistochemistry is used to guide classification.
For example, classic Hodgkin lymphoma is defined by the presence of Reed-Sternberg (RS) cells, which stain positive for the antigens CD30 and CD15 located on the cell’s membranes. In contrast, lymphocyte predominant cells, which characteristically stain positive for the antigens CD20 and CD45, are expressed by cells in lymphocyte-predominant Hodgkin lymphoma [5] . These differences in surface antigen expression have important implications for treatment selection.
Determining the stage of the disease generally involves a contrast-enhanced computed tomography (CT) scan of the neck, chest, abdomen and pelvis and (in non-Hodgkin lymphoma) a bone marrow biopsy. Recently positron emission tomography (PET) scanning has become more widely used, both as part of diagnosis and as a means of accurately assessing response to treatment.
In addition, full blood count, lactic dehydrogenase, erythrocyte sedimentation rate, liver enzymes and urea and creatinine should be checked, and patients should be screened for hepatitis B, hepatitis C and HIV. Baseline cardiac function should be checked in patients who are going to receive anthracycline-based chemotherapy (see accompanying article).
Patients with either Hodgkin lymphoma or non-Hodgkin lymphoma are classified according to the Ann Arbor staging system (see ‘Ann Arbor staging system’).
For Hodgkin lymphoma, the following categories are then used to guide treatment [5] :
- Limited stage – Ann Arbor stage 1 or 2 disease without risk factors
- Intermediate stage – Ann Arbor stage 1 or 2 disease with risk factors (e.g., age >50 years, large mediastinal mass, elevated erythrocyte sedimentation rate)
- Advanced stage – Ann Arbor stage 3 or 4 disease
Around one third of patients with Hodgkin lymphoma will have advanced stage disease at diagnosis, and their prognosis can be calculated further (see ‘Hasenclever International Prognostic Score’) [6] .
Similar scoring systems can be used to guide treatment for some non-Hodgkin lymphomas, such as the international prognostic index (IPI) [7] for diffuse large B cell lymphoma, and the follicular lymphoma international prognostic index (FLIPI) [8] for follicular lymphoma.
Stage | Involved sites |
---|---|
1 (A or B) | One lymph node region or lymphoid structure or localised extra nodal site |
2 (A or B) | Two or more lymph node regions on same side of the diaphragm |
3 (A or B) | Lymph nodes on both sides of the diaphragm |
4 (A or B) | Diffuse/disseminated extra nodal sites |
A | Absence of B symptoms |
B | Presence of B symptoms |
Age | ≥45 years |
Gender | Male |
Albumin | <40g/dl |
Haemoglobin | <10.5g/dl |
Stage | 4 |
Leucocytes | ≥15×10 /l |
Lymphocytes | <0.6×10 /l |
0 | 89% |
1 | 90% |
2 | 81% |
3 | 78% |
4 | 61% |
≥5 | 56% |
The prognosis for patients diagnosed with non-Hodgkin lymphoma in the UK has improved markedly in the past 30 years. The five-year survival rate is 63%, and half of all patients survive for at least ten years after diagnosis [1] .
However, there are marked variations in survival rates between subtypes of the disease. The most recent UK statistics indicate that 87% of patients with follicular lymphoma survive for at least five years, compared with 27% of patients with mantle cell lymphoma [1] . In general, patients with the rarer T-cell lymphomas have a poorer prognosis than patients with B-cell lymphomas.
Hodgkin lymphoma has a cure rate in the region of 80-90% [5] . The prognosis after intensive chemotherapy (with or without radiotherapy) has improved so much in recent decades that the focus has begun to move towards potentially less intensive approaches, with the aim of reducing long-term complications of therapy such as cardiac and pulmonary toxicity.
Nick Duncan MRPharmS MSc is principal pharmacist in haematology and oncology at University Hospitals Birmingham NHS Foundation Trust.
[1] Cancer Research UK. Cancer stats (Online). http://publications.cancerresearchuk.org/cancerstats (accessed 4 September 2014).
[2] Chang ET, Montgomery SM, Richiardi L et al. Number of siblings and risk of Hodgkin’s lymphoma. Cancer Epidemiology Biomarkers Prevention 2004;13:1236–1243.
[3] Swerdlow, Steven H. International Agency for Research on Cancer. WHO classification of tumours of haematopoietic and lymphoid tissues. World Health Organization classification of tumours 2 (4th ed.). Geneva: World Health Organization, 2008.
[4] Follows GA, Ardeshna KM, Barrington SF et al. Guidelines for the first line management of classical Hodgkin lymphoma. Br J Haem 2014;166:34–49.
[5] Eichenauer DA, Engert A, André M et al. Hodgkin’s lymphoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 2014;25(suppl 3):iii70–iii75.
[6] Hasenclever D, Diehl V, Armitage JO et al . A prognostic score for advanced Hodgkin’s disease. New Engl J Med 1998;339:1506–1514.
[7] The International Non-Hodgkin’s Lymphoma Prognostic Factors Project. A predictive model for aggressive non-Hodgkin’s lymphoma. New Engl J Med 1993;329:987–994.
[8] Buske C, Hoster E, Dreyling M et al. The Follicular Lymphoma International Prognostic Index (FLIPI) separates high-risk from intermediate- or low-risk patients with advanced-stage follicular lymphoma treated front-line with rituximab and the combination of cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) with respect to treatment outcome. Blood 2006;108:1504–1508.
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B-Cell Lymphoma Clinical Presentation
- Author: Mohammad Muhsin Chisti, MD, FACP; Chief Editor: Emmanuel C Besa, MD more...
- Sections B-Cell Lymphoma
- Practice Essentials
- Classification
- Pathophysiology and Etiology
- Epidemiology
- Presentation
- Approach Considerations
- Laboratory Studies
- Other Tests
- Imaging Studies
- Histologic Findings
- Immunophenotypic Analysis
- Complications
- Consultations
- Risk Stratification
- Follicular Lymphoma
- Marginal Zone Lymphomas of MALT Type
- Mantle Cell Lymphoma
- Diffuse Large B-Cell Lymphoma
- Burkitt Lymphoma
- Primary Cutaneous B-Cell Lymphomas
- Medication Summary
- Hematopoietic Growth Factors
- Monoclonal Antibodies
- Corticosteroids
- Antineoplastic Agents
- Antineoplastics, Tyrosine Kinase Inhibitor
- Antineoplastics, PI3K Delta Inhibitors
- Antineoplastics, Angiogenesis inhibitor
- PD-1/PD-L1 Inhibitors
- Antineoplastics, Anti-CD19 Monoclonal Antibodies
- CAR T-cell Therapy
- Questions & Answers
- Media Gallery
History and Physical Examination
Lymphadenopathy is the most common manifestation of lymphoma. Lymphadenopathy may wax and wane. Spontaneous remissions have been documented, most commonly in patients with low-grade lymphomas.
Systemic symptoms (B symptoms) known to be associated with adverse prognosis include the following:
- Unexplained fevers
- Night sweats
- Weight loss
Organ-specific symptoms that may lead to identification of specific sites of involvement include the following:
- Shortness of breath, chest pain, cough
- Abdominal pain and distention
- Central nervous system (CNS) symptoms
Typical physical examination findings include the following:
- Pallor (suggesting anemia)
- Purpura, petechiae, or ecchymoses (suggesting thrombocytopenia)
Findings in patients with an advanced high-grade lymphoma may include the following:
- Tachycardia
- Respiratory distress
Examples of findings that might direct further investigations and subsequent therapy include the following:
- Pharyngeal involvement
- Thyroid mass
- Evidence of pleural effusion
- Abdominal mass
- Testicular mass
- Cutaneous lesions
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- Neoplastic follicles comprising cleaved cells (centrocytes) and larger cells with vesicular nuclei and prominent 2-3 nucleoli (centroblasts).
- Mantle cell lymphoma. Small lymphoid cells with oval to slightly irregular nuclei and clumped chromatin and rare admixed pink histiocytes.
- Diffuse large B-cell non-Hodgkin lymphoma. Large cells with abundant cytoplasm and large round-ovoid nuclei with thick nuclear membrane and multiple prominent nucleoli.
- Burkitt lymphoma. Normal architecture is entirely replaced by lymphoma cells and evenly dispersed macrophages, starry sky (250×).
- Burkitt lymphoma cells with round noncleaved nuclei and strongly basophilic cytoplasm (1000×).
- Compartmentalizing fibrosis and infiltrate of medium-sized to large lymphoid cells.
- Lymphoma cells show strong membranous positivity with CD20 indicative of B-cell origin.
- Table 1. Chromosomal Abnormalities in B-Cell Non-Hodgkin Lymphoma
- Table 2. Characteristic Immunophenotypes of Major Subtypes of Lymphoma
- Table 3. Lugano Modification of the Ann Arbor Staging System.
- Table 4. Non-Hodgkin lymphoma staging.
- Table 3. International Society for Cutaneous Lymphoma/European Organization for Research and Treatment of Cancer tumor-node-metastasis classification for cutaneous B-cell lymphoma
|
|
|
|
t(14;18)(q32;q21) | Follicular, diffuse large cell | IgH* | 2 |
t(11;14)(q13;q32) | Mantle cell | IgH |
|
t(1;14)(p22;q32) | MALT lymphoma | IgH | 10 |
t(11;18)(q21;q21) | MALT lymphoma | IgH | Unknown |
t(9;14)(p13;q32) | Lymphoplasmacytic lymphoma | IgH | PAX-5 |
8q24 translocations t(8;14)(q24;q32) t(2;8)(p11-12;q24) t(8;22)(q24;q11) | Burkitt lymphoma | IgH Ig-8 Ig-6 | c- |
Trisomy 12, deletion 11q22-23, 17p13, and 6q21 | CLL | … | … |
*Immunoglobulin H (IgH) MALT = Mucosa-associated lymphoid tissue. |
|
|
Follicular | CD20 , CD3 , CD10 , CD5 , BCL2 , CD23 , CD43 , cyclinD-1 , BCL6 |
Small lymphocytic | CD 20 , CD3 , CD10 , CD5 , CD23 , |
MALT | CD20 , CD3 , CD 10 , CD5 , CD23 , BCL2 |
Marginal zone | CD20 , CD3 , CD 10 , CD5 , CD23 , CD43 , cyclinD-1 , BCL2 |
Mantle cell | CD20 , CD23 , CD10 , CD5 , CD43 , cyclinD-1+ |
Mediastinal large B-cell | CD20 , CD30+, MUM-1 |
Burkitt | slg ,CD20 , Tdt , CD10 , BCL2- , BCL6 , Ki-67 (≥95%) |
B-lymphoblastic | slg-, CD19+, CD10 , CD20 , TdT |
MALT = mucosa-associated lymphoid tissue. |
|
|
| |
I | Single node or adjacent group of nodes | Single extranodal lesions without nodal involvement | |
II | Multiple lymph node groups on same side of diaphragm | Stage I or II by nodal extent with limited contiguous extranodal involvement | |
II bulky* | Multiple lymph node groups on same side of diaphragm with “bulky disease”. | N/A | |
III | Multiple lymph node groups on both sides of diaphragm; nodes above the diaphragm with spleen involvement | N/A | |
IV | Multiple noncontiguous extranodal sites | N/A | |
*Stage II bulky disease considered limited or advanced as determined by histology and a number of prognostic factors. Suffixes A and B are not required X for bulky disease replaced with documenting of largest tumor diameter Definition of “Bulky” disease varies depending on lymphoma histology. |
|
|
| |
I | Single node or adjacent group of nodes | Single extranodal lesions without nodal involvement | |
II | Multiple lymph node groups on same side of diaphragm | Stage I or II by nodal extent with limited contiguous extranodal involvement | |
II bulky* | Multiple lymph node groups on same side of diaphragm with “bulky disease” | N/A | |
III | Multiple lymph node groups on both sides of diaphragm; nodes above the diaphragm with spleen involvement | N/A | |
IV | Multiple noncontiguous extranodal sites | N/A |
|
|
|
|
|
|
T1 | Solitary skin involvement T1a: ≤5 cm diameter T1b: >5 cm diameter | N0 | No lymph node involvement | M0 | No evidence of extracutaneous non-lymph node disease |
T2 | Multiple lesions limited to one body region or two contiguous body regions T2a: all-disease in a < 15-cm diameter T2b: all-disease in a >15- and < 30-cm diameter T2c: all-disease in a >30-cm diameter | N1 | Involvement of one peripheral lymph node region | M1 | Evidence of extracutaneous non-lymph node disease |
T3 | Generalized skin involvement T3a: multiple lesions involving two noncontiguous body regions T3b: multiple lesions involving three body regions | N2 | Involvement of two or more peripheral lymph node regions or involvement of any lymph node region that does not drain an area of current or prior skin involvement | ||
N3 | Central lymph nodes involvement |
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Contributor Information and Disclosures
Mohammad Muhsin Chisti, MD, FACP Associate Professor of Medicine (Hematology and Oncology), Oakland University William Beaumont School of Medicine; Medical Director of Research, Karmanos Cancer Institute Mohammad Muhsin Chisti, MD, FACP is a member of the following medical societies: American College of Physicians , American Medical Association , American Society of Clinical Oncology , American Society of Hematology , Medical Society of the State of New York Disclosure: Nothing to disclose.
Haresh Kumar, MBBS Resident Physician, Department of Internal Medicine, St Joseph Mercy Oakland Hospital Disclosure: Nothing to disclose.
Sumeet K Yadav, MD, MBBS Resident Physician, Department of Internal Medicine, St Joseph Mercy Oakland Hospital Sumeet K Yadav, MD, MBBS is a member of the following medical societies: American College of Physicians , Asian Medical Students' Association International , Bangladesh Medical and Dental Council , European Society for Medical Oncology , Nepal Medical Association , We Care, Jahurul Islam Medical College and Hospital Disclosure: Nothing to disclose.
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference Disclosure: Received salary from Medscape for employment. for: Medscape.
Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education , American Society of Clinical Oncology , American College of Clinical Pharmacology , American Federation for Medical Research , American Society of Hematology , New York Academy of Sciences Disclosure: Nothing to disclose.
Ajeet Gajra, MD Associate Professor of Medicine, Director of Hematology/Oncology Fellowship Program, State University of New York Upstate Medical University; Consulting Staff, Department of Internal Medicine, Division of Hematology and Oncology, Veterans Affairs Medical Center Ajeet Gajra, MD is a member of the following medical societies: American Association for Cancer Research , American Medical Association , American Society of Hematology Disclosure: Nothing to disclose.
Neerja Vajpayee, MD Associate Professor, Department of Pathology, State University of New York Upstate Medical University Neerja Vajpayee, MD is a member of the following medical societies: American Society of Hematology , College of American Pathologists , United States and Canadian Academy of Pathology Disclosure: Nothing to disclose.
The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Sara J Grethlein, MD, and Uzma Athar, MD, to the development and writing of the source article.
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Clinical presentation and characteristics of lymphoma in the head and neck region
Katharina storck.
1 Department of ENT, Head and Neck Surgery, Klinikum Rechts der Isar, Technical University of Munich, Ismaninger Strasse 22, 81675 Munich, Germany
Markus Brandstetter
Ulrich keller.
2 Third Department of Internal Medicine, Haematology and Oncology, Klinikum rechts der Isar, Technische Universität München, Ismaningerstr. 22, 81675 Munich, Germany
Andreas Knopf
Associated data.
The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.
The study analyses clinical characteristics of histologically defined head and neck (H&N) lymphoma to raise the awareness of ENT specialists to the leading symptoms.
From 2003 to 2011, all patients with histologically defined H&N lymphoma from our clinic were evaluated.
This study identified 221 patients with H&N lymphoma comprising 193 non-Hodgkin lymphomas (NHL) and 28 Hodgkin lymphomas (HL). Among NHL there were 77 indolent (iNHL), 110 aggressive (aNHL), six highly aggressive NHL and further 28 HL. Patients with highly aggressive NHL and HL were significantly younger ( p < 0.0001). Corresponding to the leading symptoms, we found nodal and extranodal involvement. NHL demonstrated manifestation in neck lymph nodes, tonsils, major salivary glands, sinonasal-system and hypopharynx/larynx. HL showed exclusive manifestation in lymph nodes of the neck and the tonsils ( p < 0.0001). The mean time from first symptoms to diagnosis ranged from 1.5 ± 0.7 months in highly aggressive lymphoma to 7.5 ± 11.5 months in iNHL.
Conclusions
The variable clinical presentation of lymphoma is a challenge for the ENT specialist. Fast diagnosis is crucial for rapid treatment, especially in highly aggressive NHL like the Burkitt-lymphoma and HL. A standardized medical history, clinical examination and imaging evaluations paired with patient’s signs, symptoms and demographic knowledge might indicate lymphoma. Biopsies in the H&N region should always be immediately performed in suspicious findings.
Lymphomas are a heterogeneous group of malignant tumours of the haematopoietic system and are characterized by the aberrant proliferation of mature lymphoid cells or their precursors [ 1 ]. Lymphomas can be divided into two major entities: Hodgkin’s lymphoma (HL) and non-Hodgkin’s lymphoma (NHL). Over 20 different subtypes of NHL have been classified according to the specific subtype of lymphoid cells involved.
Several classifications have been developed over the years for lymphomas. The currently used classification is that of the World Health Organization (WHO) and is based on the principles of the Revised European-American Classification of Lymphoid Neoplasm (REAL) from 1994 [ 2 ]. The latest update of the classification was published in two reviews in Blood in 2016 [ 3 – 5 ]. The subtype of the lymphomas is defined based on the cell of origin: B-cell lymphomas, T-cell and natural killer-cell lymphomas (T/NK-NHL) and HL [ 6 , 7 ]. The two recent WHO classifications from 2008 and 2016 include and encompass (as previously stated in previous classifications) morphology, immunophenotype, genetic and clinical features in order to define “real” diseases [ 3 , 4 ]. HLs frequently involve lymph nodes of the neck and mediastinum, whereas extranodal sites account for only 5% of HLs for example in the tonsils. In contrast, approximately 30% of NHLs show heterogeneous extranodal manifestations, such as in the major salivary glands, paranasal sinuses, mandible, maxilla and Waldeyer’s ring (largely depending and often characteristic for the specific NHL subtype) [ 3 , 8 ]. Other than the gastrointestinal tract, the head and neck region is frequently involved as an extranodal site in NHL, affecting 11–33% of patients [ 9 ]. The clinical behaviour and manifestations of lymphomas in the head and neck region usually lack specific characteristics that would enable attribution to a specific lymphoma entity without biopsy and histological evidence. In particular, with regard to lymphomas having an aggressive course, immediate histological evidence is crucial for patient management, early treatment initiation and often for the outcome [ 10 , 11 ]. Available imaging techniques (ultrasound, computed tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET)) fail to distinguish HL from NHL and cannot differentiate their various subtypes, necessitating pathological diagnosis [ 8 ]. Sometimes, clinical parameters and the various sites within the head and neck can help to distinguish between the two categories as they each have predilections as mentioned above [ 3 ]. Typical symptoms can include an indolent lymphadenopathy (here, we concentrate on the cervical lymph nodes), fatigue, occasionally B-symptoms such as fever > 38 °C, night sweats and weight loss (> 10% within 6 month), susceptibility to infections and changes in the haemogram. Especially with respect to the differential blood count, iNHL presents cytopenia more often than aNHL but it does not lead to the diagnosis as a single parameter. In chronic lymphatic leukemia (CLL), for example, the frequency of lymphocytes in the differential blood count is elevated as a characteristic sign. Further symptoms of lymphoma might include anaemia, leucopenia/leucocytosis and thrombopenia, although specific serum and blood parameters might sometimes also suggest indolent vs aggressive lymphoma, e.g. elevated lactate dehydrogenase (LDH) in cases of highly proliferative disease or increased β2-microglobulin. The most important differential diagnosis for head and neck lymphadenopathy is infection or lymph-node metastasis from regional or distant primaries being affected by solid cancer.
Our retrospective study includes 221 patients who were suffering from NHL and HL and who were consecutively diagnosed in the Department of Otorhinolaryngology, Head and Neck surgery. Histologically confirmed lymphomas were classified according to the clinical system defined below. Thorough analysis of epidemiological data, leading symptomatology, clinical disease presentation and laboratory testing were carried out to identify clinical parameters in order to expedite diagnostic regimes/work-up.
Patients and methods
All patients presenting with head and neck symptoms that resulted in the histologically established diagnosis of lymphoma from January 2003 to December 2011 ( n = 221) were included in this retrospective study. The study has been approved by the ethic committee of the Technical University of Munich (Permit Number: 493/17).
A standardized medical history was obtained from all patients: clinical examination, age at diagnosis, gender, location in the head and neck region, imaging evaluations (especially ultrasound), leading symptoms (B-symptoms; fever, night sweats, weight loss), time to diagnosis, known risk factors (HIV, EBV), histological findings and survival outcome (Munich cancer centre). All patients underwent clinical examination and a high-resolution B-mode ultrasound of the neck (S2000, tissue harmonic imaging, 9 MHz linear array, Siemens, Germany). Blood chemistry (including LDH and C-reactive protein (CRP)) and a complete blood count with a leucocyte differential count were undertaken. During diagnostic work-up (staging), all patients also underwent contrast CT-scans of the neck, chest, abdomen and pelvic cavity and bone marrow biopsy. If central nervous system involvement was suspected, staging also included contrast MR imaging of the brain and/or the spine.
Lymphomas were classified according to the lymphoma classification effective at the time: up until 2008, we used the Revised European American Lymphoma Classification (REAL) [ 2 ], and starting from 2008, we employed the WHO classification [ 3 , 4 ]. For practical purposes and because the observation period spanned two classifications, we refer here to lymphomas in two main categories, namely Hodgkin lymphomas (HL) and non-Hodgkin lymphomas (NHL). NHL were further clinically subgrouped into 1. indolent lymphoma (iNHL) (including follicular lymphomas and margional zone lymphomas), 2. aggressive lymphoma (aNHL) (e.g. DLBCL) and 3. highly aggressive lymphoma (Burkitt lymphoma and lymphoblastic lymphomas) (Fig. (Fig.1 1 ).
![Click on image to zoom An external file that holds a picture, illustration, etc.
Object name is 13005_2018_186_Fig1_HTML.jpg](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6317257/bin/13005_2018_186_Fig1_HTML.jpg)
Frequency of histologic subtypes within the iNHL, aNHL and highly aggressive lymphoma and HL
Statistical analyses were performed by using unpaired t-tests and one-way ANOVA testing (SPSS Inc., Chicago, IL). Post-hoc analysis was carried out with Tukey’s test. A p value of < 0.05 was considered statistically significant and a p value < 0.001 was defined as highly significant.
Epidemiology and characteristics of the head and neck cohort
A total of 221 patients were included in this study: 193 with NHL and 28 with HL. With respect to the clinical classification system, we included 77 indolent NHL (iNHL), 110 aggressive lymphomas (aNHL), 6 highly aggressive lymphomas and 28 HL (Fig. (Fig.1). 1 ). The median age for indolent and for aggressive lymphoma was 70 years, for highly aggressive lymphoma 34 years and for HL 33 years. Patients with highly aggressive lymphoma and HL were significantly younger than their counterparts with less aggressive types ( p < 0.0001; Table Table1). 1 ). The study comprised 114 [52%] males and 107 [48%] females without differences between the groups (Table (Table1 1 ).
Frequency of histologic types of head and neck lymphoma including also epidemiology, symptomatology, disease manifestations, localization and laboratory findings
Indolent lymphoma | Aggressive lymphoma | Highly aggressive lymphoma | Hodgkin lymphoma | -value | |
---|---|---|---|---|---|
n | 77 | 110 | 6 | 28 | |
Age | < 0.0001 | ||||
Mean ± SD | 72 ± 39 | 66 ± 16 | 41 ± 24 | 41 ± 21 | |
Median | 70 | 70 | 34 | 33 | |
Gender, n [%] | 0.11 | ||||
Male | 37 [48] | 57 [52] | 6 [100] | 14 [50] | |
Female | 40 [52] | 53 [48] | 0 | 14 [50] | |
First Diagnosis, n [%] | 62 [81] | 90 [82] | 5 [83] | 27 [96] | 0.26 |
Time to diagnosis, [Months] | 0.22 | ||||
Mean ± SD | 7.5 ± 11.5 | 3.7 ± 8.5 | 1.5 ± 0.7 | 3.4 ± 3.5 | |
Median | 3.0 | 2.0 | 1.5 | 1.5 | |
Leading symptom | 0.045 | ||||
Cervical mass | 62 [81] | 76 [69] | 3 [100] | 27 [96] | |
Globus pharyngis | 3 [4] | 6 [5] | 0 | 0 | |
Odyno−/dysphagia | 7 [9] | 21 [19] | 3 [100] | 1 [4] | |
Dysphonia | 0 | 1 [1] | 0 | 0 | |
Dyspnea | 2 [3] | 3 [3] | 0 | 0 | |
Incidentally | 2 [3] | 3 [3] | 0 | 0 | |
Localization, n [%] | < 0.0001 | ||||
Major salivary gland | 21 [27] | 12 [11] | 0 | 0 | |
Sinonasal system | 2 [3] | 4 [4] | 0 | 0 | |
Tonsils | 15 [20] | 41 [37] | 3 [50] | 1 [4] | |
Hypopharynx/Larynx | 1 [1] | 6 [6] | 0 | 0 | |
Lymph node | 37 [48] | 43 [39] | 3 [50] | 27 [96] | |
Other | 1 [1] | 4 [4] | 0 | 0 | |
Laterality, n [%] | 0.62 | ||||
Unilateral | 65 [85] | 90 [82] | 6 [100] | 22 [79] | |
Bilateral | 12 [15] | 20 [18] | 0 | 6 [21] | |
Systemic disease, n [%] | 43 [56] | 75 [68] | 6 [100] | 22 [79] | 0.90 |
B-symptoms, n [%] | 5 [7] | 20 [18] | 0 | 3 [11] | 0.30 |
Laboratory parameter, Mean ± SD | |||||
Leucocytes | 11.8 ± 12.0 | 7.7 ± 3.6 | 4.5 ± 3.2 | 8.0 ± 3.0 | 0.043 |
Hemoglobin | 13.6 ± 1.9 | 13.4 ± 2.3 | 10.4 ± 6.1 | 13.0 ± 2.9 | 0.24 |
CRP | 1.53 ± 2.88 | 1.80 ± 3.00 | 1.20 ± 2.00 | 3.30 ± 4.30 | 0.15 |
LDH | 263 ± 285 | 278 ± 291 | 232 ± 229 | 271 ± 121 | 0.97 |
aNHL Aggressive non-Hodgkin lymphoma, haNHL Highly aggressive non-Hodgkin lymphoma, HL Hodgkin lymphoma, iNHL Indolent non-Hodgkin lymphoma
The mean time from first symptoms to diagnosis ranged from 1.5 ± 0.7 months in highly aggressive lymphoma to 7.5 ± 11.5 months in indolent lymphoma. This difference was not statistically significant (Table (Table1 1 ).
Independent from the classification the vast majority of lymphoma patients ( n = 168) suffered from cervical masses as the leading symptom. Fifty-nine patients complained of odyno−/dysphagia. Globus pharyngis, dysphonia and dyspnea occurred infrequently. Occult lymphoma without clinical symptoms was diagnosed in five patients during sonographic procedure for another disease (Table (Table1). 1 ). The distribution of leading symptoms differed significantly between the groups ( p < 0.05, Table Table1). 1 ). Whereas patients with highly aggressive lymphoma and HL usually presented with a cervical mass and/or odyno/dysphagia, patients with indolent and aggressive lymphoma demonstrated a broad variety of leading symptoms (Table (Table1). 1 ). B-symptoms occurred in 28 (13%) patients (NHL, n = 25; HL, n = 3).
Disease manifestation
Corresponding to the diverse leading symptoms, we found a nodal and an extranodal involvement of the head and neck organs. NHL demonstrated manifestation in neck lymph nodes ( n = 83), tonsils ( n = 60), major salivary glands ( n = 32), the sinonasal system ( n = 6) and the hypopharynx/larynx ( n = 7) whereas HL showed exclusive manifestation in neck lymph nodes ( n = 27) and the tonsils ( n = 1). Although highly aggressive NHL and HL exclusively originated in indolent neck lymph nodes and the tonsils, indolent and aggressive lymphomas showed a distinct disease heterotopia ( p < 0.0001; Table Table1). 1 ). In our study, extranodal head and neck manifestation occurred in 57% NHL and in 4% HL. NHL presented a unilateral localization in 84% of cases, and HLs were unilateral in 79% of cases. We found n = 12 cases (20%) of NHL with bilaterally affected tonsils. Systemic involvement was seen in n = 43 [56%] patients with iNHL, in n = 75 [68%] patients with aNHL, in n = 6 [100%] patients with highly aggressive lymphoma, and in n = 22 [79%] patients with HL.
![](http://sektorel.online/777/templates/cheerup/res/banner1.gif)
Laboratory findings
Basic laboratory testing included blood counts, C-reactive protein (CRP) and lactate dehydrogenase (LDH). The level of leucocytes (normal range: 4.0–9.0 [G/l]) differed significantly between the groups ( p < 0.05; Table Table1). 1 ). However, all levels ranged within the norm. Patients with iNHL exhibited a leucocyte level of 11.8 ± 12.0 (mean ± SD), patients with aNHL 7.7 ± 3.6 and patients with HL 8.0 ± 3.0. In patients with highly aggressive lymphoma, the leucocyte level was significantly decreased at 4.5 ± 3.2. Similar results were seen in haemoglobin levels, which showed a normal level in all the groups, with the lowest level in highly aggressive lymphomas at 10.5 ± 6.1 (mean ± SD). Differences between haemoglobin levels were not statistically significant. CRP (normal range: < 0.5 [mg/dl]) was slightly elevated in all groups, with the highest level in HL at 3.30 ± 4.30 (mean ± SD). LDH levels (normal range: < 244 [U/l]) were elevated in aNHL at 278 ± 291[U/l] (mean ± SD) and in HL at 271 ± 121[U/l] (Table (Table1 1 ).
Survival outcome
Available overall survival data for the previously defined subgroups are shown in Fig. Fig.2 2 .
![Click on image to zoom An external file that holds a picture, illustration, etc.
Object name is 13005_2018_186_Fig2_HTML.jpg](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6317257/bin/13005_2018_186_Fig2_HTML.jpg)
Overall survival data for the previously defined subgroups
Using the cox’s regression for forward selection, we also evaluated the survival rate depending on the laboratory findings. We could not find any significant differences in the survival rate depending on (pathological) laboratory findings (Fig. (Fig.3 3 ).
![Click on image to zoom An external file that holds a picture, illustration, etc.
Object name is 13005_2018_186_Fig3_HTML.jpg](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6317257/bin/13005_2018_186_Fig3_HTML.jpg)
Survival rate depending on the laboratory findings
Lymphoma is the third most common malignancy worldwide representing 3% of all malignant tumours. With 12% of all malignant tumours of the head and neck region, lymphomas are the third most frequent malignancy after squamous cell carcinoma (46%) and thyroid carcinoma (33%) [ 12 , 13 ] and should thus always be taken into consideration in cases of unknown cervical or oral masses. Misinterpretation of the clinical appearance and of the radiological findings (ultrasound, CT-scan, MRI) can lead to delay in diagnosis, delayed treatment initiation and impairment of the patient’s prognosis. In the current study, we have analysed clinical and epidemiological data of the entire lymphoma cohort diagnosed within an eight-year time frame at our ENT Department in order to bring to the attention of ENT specialists the specific clinical symptoms that allow the early diagnosis of lymphomas. In agreement with recent publications, we saw no differences in the gender distribution. Patients with HL (33 years) and highly aggressive lymphomas (34 years) were significantly younger than patients having other lymphoma subtypes (70 years) [ 14 , 15 ]. In our study, we found 193 NHL and 28 HL. Concordant with the present literature, diffuse large B-cell NHL (DLBCL) comprised the largest percentage of NHL in the head and neck region with 36.7% of cases [ 16 , 17 ]. Cervical lymphadenopathy (syn. nodal) is the most common site for both NHL and HL in the head and neck region. Differentiation from other causes of pathological lymph node enlargement caused by infectious diseases (CMV, EBV) or metastatic squamous cell carcinoma is crucial and often difficult and requires histopathological assessment. Certain differences, including the history of alcohol and / or tobacco use, the age of the patient, abnormalities in the clinical ENT examination, constitutional symptoms and systemic lymphadenopathy, may increase the probability of one versus the other. Associated mediastinal adenopathy is more common in HL and abdominal adenopathy in NHL [ 15 ]. In our series, we found n = 110 (50%) patients presenting with cervical lymphadenopathy. Of these, 90 were unilateral and only 20 presented with a bilateral cervical lymphadenopathy. In HL, 22/27 were unilateral. An important aspect for ENT and maxillofacial specialists is the variety of extranodal sites. Taking all lymphomas together, we found 111 (50%) lymphomas in extranodal sites. In particular, NHL contributed to this high proportion. With n = 110 of 193 NHL (57%), NHL presented an extranodal site in a surprisingly large number of cases. In the literature, 25–30% NHL occur in extranodal sites [ 18 ]. In HL, we found an extranodal site in just one case (palatine tonsils), whereas all other patients presented with cervical lymph nodes (96%). The literature also describes > 90% manifestations of HL occurring in the lymph nodes and only 1–4% involving extranodal areas [ 8 , 14 , 19 ]. The extranodal sites included in this study were the major salivary glands ( n = 33), sinonasal system ( n = 6), palatinal tonsils/nasopharynx ( n = 60) and hypopharynx/larynx ( n = 7). The literature also describes extranodal sites such as the palate, buccal mucosa, maxilla and mandible [ 8 , 20 ]. In our clinic, the patients with lymphomas in bone regions usually attend the Department of Maxillofacial Surgery; thus, patients with extranodal sites are partially preselected. The leading symptoms were correlated with the localization of the tumour mass, the majority of patients presenting with a cervical mass (76%) followed by odyno−/dysphagia, globus pharyngis, dysphonia and dyspnea.
Taking all patients together ( n = 221), we found that only n = 8 (13%) of the patients presented with constitutional symptoms or specific B-symptoms. Only n = 3 patients with HL suffered from B-symptoms. This low percentage agrees with the data in the literature [ 21 , 22 ]. Concentration on the presence or absence of B-symptoms might thus mislead the physician, as the rate of patients without such symptoms is high. The same also applies to results from blood and serum testing, as the majority of all of our patients had normal haemoglobin and leucocyte counts and only a few had slightly elevated levels of LDH and CRP. According to the WHO classification, two major subtypes of NHL (DLBCL 70–80% and Burkitt 7–20%) are related to HIV [ 23 ]. In our cohort, we found two HIV-positive patients. One of them was diagnosed with a nodal DLBCL and the other with nodal plasmablastic lymphoma (PBL), an aggressive and rare DLBCL subtype that is commonly found in patients with HIV [ 24 ].
Burkitt lymphoma (BL) is listed in the WHO’s classification of lymphoid tumours as an “aggressive B-cell non-Hodgkin’s lymphoma” characterized by a high degree of proliferation of malignant cells and deregulation of the MYC gene [ 25 ]. In our study, we found 5 cases of BL and all were male, as reported in the literature [ 20 ]; none of them were associated with HIV or EBV [ 26 ]. With a median age of 34 years, these patients were significantly younger ( p < 0.0001) than patients suffering from iNHL or aNHL. Only 1.2% of BL are of extranodal origin in the head and neck [ 27 ]. We found three to be extranodal in the tonsils. The median time to diagnosis was 1.5 months. Despite its highly aggressive nature, BL is a curable lymphoma. Patients have a better prognosis when the diagnosis is established rapidly and if they present with a limited stage [ 28 ]. BL patients exhibited the lowest leucocyte and haemoglobin levels but the levels still ranged within the norm and the number of patients was too low to make a statistically valid statement. Patients with highly aggressive lymphomas and HL all presented with either a cervical mass as a sign of a nodal lymphoma or odynophagia / dysphagia, with the tonsils as the extranodal site in all cases, reflecting their admission to the ENT Department. These findings were highly significant compared with iNHL and aNHL aggressive lymphomas with a larger variety of localizations and leading symptoms. All six highly aggressive lymphomas showed a unilateral cervical mass but a systemic dissemination.
All Patients received standard therapies through the hematology department or associated hematologists/oncologist.
The focus of this report is to describe the different clinical presentations of lymphomas in the head and neck region and to raise awareness on the wide variety of symptoms. As we included all types of lymphoma that may manifest in the head and neck region, there is a large variety standard therapy approaches which sometimes were also adapted to comorbidity. Thus, the intend of this report was not to focus on this clearly important issue which is covered by numerous publications and results from prospective studies. Furthermore, treatment standards have evolved during the observation time of the patient groups described herein.
Concerning the survival outcome, we could see significant differences between the groups as expected. In the blood and serum testings we did not see any differences concerning the survival rate, which strengthens our assumption that we can not identify lymphomas in the head and neck region only by laboratory findings.
Lymphomas comprise 12% of all head and neck malignancies. The variable clinical presentation of lymphoma, in addition to the nodal involvement, is sometimes a challenge for the ENT specialist. A rapid diagnosis is crucial for early treatment initiation, especially in cases of BL and HL, which mostly affect younger patients. A standardized medical history, clinical examination and imaging evaluations (especially ultrasound) paired with patient’s signs, symptoms and demographic knowledge (e.g. age, gender, HIV, EBV) may lead to a correct diagnosis and accelerated the decision for a biopsy.
Tumours in the head and neck are easily accessible and a biopsy should immediately be performed following suspicious findings. In particular, for NHL with extranodal involvement in the head and neck occurring at a frequency of 20–30%, biopsy should always be part of the diagnosis in any head and neck lesion, including those in the oral cavity, major salivary glands, oropharynx, nasopharynx, paranasal sinus and larynx. Unilaterality, the absence of EBV or other acute viruses, the absence of an obvious tumour and a systemic involvement (if previously noted at the first presentation) should alert the ENT specialist to lymphomas, even in the absence of B-symptoms or blood disturbances.
Acknowledgments
This work was supported by the German Research Foundation (DFG) and the Technical University of Munich within the funding programme Open Access Publishing.
The corresponding author states no financial or other relationships with other people or organizations, which may lead to a conflict of interest.
Availability of data and materials
Authors’ contributions.
Conceived and designed the study: KS, AK, MB. Performed the study and analysed the data: KS, MB, UK, AK. Wrote the paper: KS. All authors read and approved the final manuscript.
Ethics approval and consent to participate
The study has been approved by the ethic committee of the Technical University of Munich (Permit Number: 493/17).
Consent for publication
Not applicable
Competing interests
The authors declare that they have no competing interests.
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Contributor Information
Katharina Storck, Phone: 0049-89-4140-2370, Email: moc.oohay@kcrots_anirahtak .
Markus Brandstetter, Phone: 0049-89-4140-2370, Email: [email protected] .
Ulrich Keller, Phone: 0049-89-4140-4111, Email: [email protected] .
Andreas Knopf, Phone: 0049-89-4140-2370, Email: [email protected] .
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Hematologist Stephen Ansell, M.D., answers the most frequently asked questions about lymphoma.
Well, many times we don't actually know. We do know what exactly happens in the cells. We can see that the cells undergo a genetic change. And as they do that, they may grow quicker than they should, and they may persist and not die off like they should. That causes them to slowly accumulate over time. But exactly what brought about that genetic change, we don't always know.
This is not a disease that's passed down in families, although families can be more susceptible. But we think there are some susceptibility genes that may put you at risk for being more likely to get lymphoma. That does, however, require something else to happen, often in the way of exposures to toxins or viruses or something else.
Well, I think it's important to recognize what the goals of treatment are. Low-grade lymphomas have an advantage in that they can take a very long time to cause any symptoms, and certainly a very long time to put the patient's health at risk. However, we do not have a curative treatment that will fix the cancer right away. So we want to weigh up the potential risks and side effects that come with treatment compared to, clearly, the risks and side effects that come from the cancer. So, if you have a cancer that is very low-grade, growing very slowly, giving you no symptoms, we would hold off on treatment and only initiate it when you truly need it.
Well, important to know that chemotherapy may have two components. Chemotherapy, or chemical drugs that are targeting the cancer, immunotherapy, or antibody treatments that are going after proteins that are on the outside of the cancer or lymphoma cells. The goal of chemotherapy is to kill quickly- growing cells, which is a good thing because lymphoma, many times, those cells are growing quickly. The challenge, however, is there are healthy cells that may also be growing quickly. Immunotherapy, as I mentioned, binds or attacks proteins on the outsides of cells. But some of the lymphoma cells and some of the normal cells have the same proteins. So those cells may be depleted, and your immune system may become a little bit more suppressed as one of the potential side effects of therapy.
Well, I really wish that was true. Unfortunately, that's not exactly correct. There isn't a treatment or exercise program that directly targets or goes after the lymphoma cells. Generally, however, what a healthy balanced diet and a good exercise program is doing is improving your general well-being, improving your immune system function, and allowing you to tolerate the chemotherapy and fight against the cancer to a greater degree. The good news is that many studies have shown that a healthy patient who's in good shape actually has a better outcome when receiving treatment for lymphoma. So that's a strong motivation for you to be healthy by eating well and exercising regularly.
Get as much information as you can. Partner with your physician, your nurse practitioner, your PA and other members of the team and ask questions. The goal moving forward is for you to have the best outcome possible. So that sharing of information between your team and you is critical to your outcome and the best results we could hope for.
![typical presentation of lymphoma A needle suctioning out liquid bone marrow from hipbone](https://www.mayoclinic.org/-/media/kcms/gbs/patient-consumer/images/2014/02/24/12/19/r7_bonemarrowaspiration.jpg)
Bone marrow exam
In a bone marrow aspiration, a healthcare professional uses a thin needle to remove a small amount of liquid bone marrow. It is usually taken from a spot in the back of the hipbone, also called the pelvis. A bone marrow biopsy is often done at the same time. This second procedure removes a small piece of bone tissue and the enclosed marrow.
Lymphoma diagnosis often begins with an exam that checks for swollen lymph nodes in the neck, underarm and groin. Other tests include imaging tests and removing some cells for testing. The type of tests used for diagnosis may depend on the lymphoma's location and your symptoms.
Physical Exam
A healthcare professional may start by asking about your symptoms. The health professional also may ask about your health history.
Next, the healthcare professional may feel and press on parts of your body to check for swelling or pain. To find swollen lymph nodes, the health professional may feel your neck, underarm and groin. Be sure to say if you have felt any lumps or pain.
A biopsy is a procedure to remove a sample of tissue for testing in a lab. For lymphoma, the biopsy typically involves removing one or more lymph nodes. The lymph nodes go to a lab for testing to look for cancer cells. Other special tests give more details about the cancer cells. Your healthcare team will use this information to make a treatment plan.
Imaging tests
Your healthcare team may recommend imaging tests to look for signs of lymphoma in other areas of your body. Tests may include CT, MRI and positron emission tomography scans, also called PET scans.
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Many types of treatments exist for lymphoma. Treatments include radiation, chemotherapy, immunotherapy, targeted therapy and bone marrow transplant, also called stem cell transplant. Sometimes, a combination of treatments is used. The treatment that's best for you will depend on the kind of lymphoma that you have.
Treatment might not need to start right away
Treatment for lymphoma doesn't always need to start right away. Some types of lymphoma grow very slowly. You and your healthcare professional may decide to wait and have treatment if the cancer starts to cause symptoms.
If you don't have treatment, you'll have regular appointments with your healthcare professional to monitor symptoms.
Chemotherapy
Chemotherapy treats cancer with strong medicines. Most chemotherapy medicines are given through a vein. Some come in pill form. Two or more of these medicines together are often used to treat lymphoma.
Immunotherapy
Immunotherapy for cancer is a treatment with medicine that helps the body's immune system to kill cancer cells. The immune system fights off diseases by attacking germs and other cells that shouldn't be in the body. Cancer cells survive by hiding from the immune system. Immunotherapy helps the immune system cells find and kill the cancer cells. It can be given for different types of lymphoma.
Targeted therapy
Targeted therapy for cancer is a treatment that uses medicines that attack specific chemicals in the cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die. Your lymphoma cells might be tested to see if targeted therapy will help you.
Radiation therapy
Radiation therapy treats cancer with powerful energy beams. The energy comes from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points in your body.
CAR-T cell therapy
Chimeric antigen receptor (CAR)-T cell therapy, also called CAR-T cell therapy, trains your immune system cells to fight lymphoma. This treatment begins with removing some white blood cells, including T cells, from your blood. The cells are sent to a lab. In the lab, the cells are treated to recognize the lymphoma cells. The cells are then put back into your body. They then can find and destroy the lymphoma cells.
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Clinical trials
Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition.
Alternative medicine
No alternative medicines have been found to treat lymphoma. But integrative medicine may help you cope with the stress of a cancer diagnosis and the side effects of cancer treatment.
Talk to your healthcare professional about your options, such as:
- Acupuncture.
- Art therapy.
- Meditation.
- Music therapy.
- Physical activity.
- Relaxation exercises.
Coping and support
A lymphoma diagnosis can be overwhelming. With time you'll find ways to cope with the stress and uncertainty that often comes with a lymphoma diagnosis. Until then, you may find that it helps to:
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Learn about lymphoma.
If you'd like to know more about your lymphoma, ask your healthcare professional for the details of your cancer. Ask about the type and your prognosis. Ask for good sources of up-to-date information on your treatment options. Knowing more about your cancer and your options may help you feel more confident when making treatment decisions.
Keep your friends and family close
Your friends and family can be emotional support and provide the practical support you'll need, too, such as helping take care of your house if you're in the hospital.
Find someone to talk with
Find a good listener with whom you can talk about your hopes and fears. This may be a friend or a family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group also may be helpful. Ask your healthcare professional about support groups in your area. You also might contact a cancer organization such as the National Cancer Institute or the Leukemia & Lymphoma Society.
Preparing for your appointment
Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you. If your healthcare professional suspects that you have lymphoma, that person may refer you to a doctor who specializes in diseases that affect the blood cells. This kind of doctor is called a hematologist.
Appointments can be brief, and there's a lot to discuss. It's a good idea to be prepared. Here's how to help get ready and what to expect:
What you can do
- Be aware of any pre-appointment restrictions. When you make the appointment, ask if you need to do anything in advance, like restrict your diet.
- Write down any symptoms you're experiencing, even any that may seem unrelated to why you scheduled the appointment.
- Write down key personal information, including any major stresses or recent life changes.
- Make a list of all medications, vitamins or supplements you're taking.
- Consider taking a family member or friend along. Sometimes it can be difficult to remember all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
- Write down questions to ask your healthcare professional.
Your time with your healthcare professional is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For lymphoma, some basic questions to ask include:
- Do I have lymphoma?
- What type of lymphoma do I have?
- What stage is my lymphoma?
- Is my lymphoma aggressive or slow growing?
- Will I need more tests?
- Will I need treatment?
- What are my treatment options?
- What are the potential side effects of each treatment?
- How will treatment affect my daily life? Can I continue working?
- How long will treatment last?
- Is there one treatment that you feel is best for me?
- If you had a friend or loved one in my situation, what advice would you give that person?
- Should I see a lymphoma specialist? What will that cost, and will my insurance cover it?
- Do you have brochures or other printed material that I can take with me? What websites do you recommend?
Ask any other questions that come to mind during your appointment.
What to expect from your doctor
Your healthcare professional is likely to ask you several questions. Being ready to answer them may allow more time to cover other points you want to address. Your healthcare professional may ask:
- When did you first experience symptoms?
- Are your symptoms continuous or occasional?
- How severe are your symptoms?
- What, if anything, helps improve your symptoms?
- What, if anything, worsens your symptoms?
- Has anyone in your family had cancer, including lymphoma?
- Have you or has anyone in your family had immune system conditions?
- Have you or your family been exposed to toxins?
- Lymphoma – Non-Hodgkin. Cancer.Net. https:www.cancer.net/cancer-types/41246/view-all. Accessed Dec. 18, 2023.
- Lymphoma – Patient version. National Cancer Institute. https://www.cancer.gov/types/lymphoma. Accessed Dec. 18, 2023.
- What causes non-Hodgkin lymphoma? American Cancer Society. https://www.cancer.org/cancer/types/non-hodgkin-lymphoma/causes-risks-prevention/what-causes.html. Accessed Dec. 21, 2023.
- Elsevier Point of Care. Clinical Overview: Hodgkin lymphoma. https://www.clinicalkey.com. Accessed Dec. 21, 2023.
- Freedman A, et al. Clinical presentation and initial evaluation of non-Hodgkin lymphoma. https://www.uptodate.com/contents/search. Accessed Jan. 30, 2024.
- Lymphoma – Non-Hodgkin: Diagnosis. https://www.cancer.net/cancer-types/lymphoma-non-hodgkin/diagnosis. Accessed Dec. 22, 2023.
- Non-Hodgkin lymphoma treatment (PDQ) – Patient version. National Cancer Institute. https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq. Accessed Jan. 1, 2024.
- Brondfield S, et al. Developing a community for patients with cancer through longer-term art therapy. Oncology Practice. 2020; doi:10.1200/OP.20.00419.
- Distress management. National Comprehensive Cancer Network. https://www.nccn.org/guidelines/guidelines-detail?category=3&id=1431. Accessed Jan. 3, 2024.
- Coping with cancer. Cancer.Net. https://www.cancer.net/coping-with-cancer. Accessed Jan. 4, 2024.
- Robetorye R, et al. Incorporation of digital gene expression profiling for cell-of-origin determination (Lymph2Cx Testing) into the routine work-up of diffuse large B-Cell lymphoma. Journal of Hematopathology. 2019; doi:10.1007/s12308-019-00344-0.
- Laurent C, et al. Impact of expert pathologic review of lymphoma diagnosis: Study of patients from the French Lymphopath Network. Journal of Clinical Oncology. 2017; doi:10.1200/JCO.2016.71.2083.
- Member institutions. Alliance for Clinical Trials in Oncology. https://www.allianceforclinicaltrialsinoncology.org/main/public/standard.xhtml?path=%2FPublic%2FInstitutions. Accessed April 14, 2024.
- Membership institution lists. NRG Oncology. https://www.nrgoncology.org/About-Us/Membership/Member-Institution-Lists. Accessed April 14, 2024.
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Presentation, staging and diagnosis of lymphoma: a clinical perspective
Affiliation.
- 1 Department of Physiology, Fatima Jinnah Dental College, Karachi, Pakistan. [email protected]
- PMID: 19999217
Background: Due to lack of awareness among health professionals, lymphoma is often misdiagnosed. This study was done to evaluate the clinical features and histopathologic subtypes of lymphoma.
Methods: Sixty diagnosed cases of lymphoma were selected (aged 12-65 years) from medical units of Civil Hospital Karachi, during 1993 to 1998. Clinical history, physical examination and basic laboratory investigations including imaging procedures were done in all the patients. The diagnosis of lymphoma was based on histology, following the International Working Formulation classification system. This included lymph node biopsy and in some cases, biopsy of the bone marrow. The Ann Arbor Staging Classification was used to classify the extent of disease.
Results: Out of 60 cases of lymphoma, 81.6% (49 cases) were diagnosed as non-Hodgkin's lymphoma and 18.3% (11 cases) as Hodgkin's disease, with an overall male predominance. Both categories exhibited a bimodal age distribution. Lymphadenopathy was the commonest presenting features in both the types of lymphomas; however, patients with Hodgkin's disease had a prominence of 'B' symptoms, whereas abdominal signs and symptoms were more common in non-Hodgkin's lymphoma. On histopathology, majority of non-Hodgkin's lymphomas (91.8%) showed a diffuse pattern, while mixed cellularity was the commonest type seen in Hodgkin's disease (81.8%).
Conclusion: Non-Hodgkin's lymphoma was 4 times more common than Hodgkin's disease. The vast clinical spectrum of lymphoma sometimes delays its diagnosis, leading to its eventual presentation in late stages. A general awareness is hence required among the health professionals regarding its varied clinical presentations.
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Primary cauda equina lymphoma mimicking meningioma.
![typical presentation of lymphoma typical presentation of lymphoma](https://www.mdpi.com/profiles/1752921/thumb/Pierfrancesco_Lapolla.jpg)
1. Introduction
2.1. clinical neurological presentation, 2.2. imaging findings and additional diagnostics, 2.3. operative findings and histopathology, 2.4. postoperative recovery, 2.5. management in the infectious diseases department and follow-up, 2.6. subsequent oncological treatment, 2.7. long-term follow-up and prognosis, 3. materials and methods.
- Intravenous chemotherapy: administered to 27% of patients, aimed at systemic disease control;
- Radiotherapy: applied in 44% of cases, targeting the tumor localized within the spinal canal;
- Surgical intervention: cytoreductive surgeries performed in 11% of cases, aimed at decompressing neural structures and alleviating symptoms.
5. Discussion
5.1. clinical presentation, 5.2. diagnostic approaches, 5.3. differential diagnosis, 5.4. histopathological examination and diagnosis, 5.5. management and prognosis, 5.6. comparative analysis, 5.7. limitations, 6. conclusions, author contributions, institutional review board statement, informed consent statement, data availability statement, acknowledgments, conflicts of interest.
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Case | Authors, Year (y) | Age (y) | Sex | Clinical Presentation | Management | Outcome |
---|---|---|---|---|---|---|
1 | Mauney et al., 1983 [ ] | 68 | F | Paraplegia, flaccid paralysis of lower extremities with muscular atrophy, deep tendon reflexes absent, and sensory loss up to the mid-thigh without return of bowel or bladder. | Myelogram, cerebrospinal fluid examination, bilateral laminectomy at T12-L3 | Follow-up 3 months: no improvement of flaccid paralysis nor return of bowel or bladder |
2 | Toner et al., 1987 [ ] | 59 | M | CES | Biopsy, myelogram, cerebrospinal fluid examination | Alive at 22 months |
3 | Klein et al., 1990 [ ] | 29 | F | CES (AIDS) | Tumor resection | Fulminant lymphomatous meningitis and died |
4 | Giobbia et al., 1999 [ ] | 30 | F | Cauda equina irritation | Cytology of CSF, RT treatment | Alive at 12 months |
5 | Zagami and Granot, 2003 [ ] | 71 | F | Cauda equina irritation | 8th thoracic laminectomy, CSF, intrathecal chemotherapy | Dead at 8 months |
6 | Tajima et al., 2007 [ ] | 67 | F | Polyradiculoneuropathy | Biopsy, CSF intrathecal chemotherapy; RT | Alive at 36 months |
7 | Khong et al., 2008 [ ] | 16 | M | CES | Biopsy, intravenous chemotherapy; RT | Alive at 12 months |
8 | Beitzke et al., 2010 [ ] | 69 | M | Pronounced flaccid lower extremity paraparesis and severe low-back pain | Biopsy, positron emission tomography, cerebrospinal fluid examination, contrast-enhanced MRI | Died of septic shock soon after diagnosis |
9 | Teo et al., 2012 [ ] | 58 | M | CES | Biopsy, intrathecal chemotherapy; RT | Alive at 24 months |
10 | Cugati et al., 2012 [ ] | 11 | M | CES | Tumor excision, intrathecal chemotherapy; RT | Alive at 12 months |
11 | Iwasaki et al., 2012 [ ] | 69 | M | CES | Biopsy, PET/CT, intravenous chemotherapy; RT | Dead at 18 months |
12 | Nishida et al., 2012 [ ] | 47 | M | CES | CSF, PET/TC, intravenous and intrathecal chemotherapy, RT | Alive at 18 months |
13 | Nakashima et al., 2014 [ ] | 59 | M | CES | Biopsy, chemotherapy, RT | Alive at 12 months |
14 | Broen et al., 2014 [ ] | 71 | F | S1 radiculopathy | Biopsy, PET/TC intravenous and intrathecal chemotherapy | Alive at 10 months |
15 | Broen et al., 2014 [ ] | 75 | F | L5 radiculopathy | Biopsy, steroid only | Dead after 11 months |
16 | Shin et al., 2016 [ ] | 79 | F | CES | Biopsy | Alive at final follow-up |
17 | Belcastro et al., 2016 [ ] | 47 | M | CES | Biopsy, intrathecal chemotherapy | Dead 2 months, |
18 | Suzuki et al., 2018 [ ] | 65 | M | Progressive motor palsy in the legs and gait disturbance over the last 5 months; deep tendon reflexes of the bilateral lower extremities were diminished. Sensory disturbance was also found in the bilateral lower extremities. | Biopsy after laminectomy, positron emission tomography, cerebrospinal fluid examination, contrast-enhanced MRI, cytarabine and methotrexate (high dose) | Complete remission after 6 years |
Case | Authors, y | Neuroradiological Finding | Histological Classification | Side |
---|---|---|---|---|
1 | Mauney et al., 1983 [ ] | Multiple intradural masses with a complete block of the subarachnoid space | B-cell malignant large-cell noncleaved (Lukes and Collins classification) | T12-L3 |
2 | Toner et al., 1987 [ ] | Intradural masses with a complete | B-cell large-cell | Lumbar nerve root |
3 | Klein et al., 1990 [ ] | Swelling of cauda equina, T1WI focally high | - | L1-L2 |
4 | Giobbia et al., 1999 [ ] | Enhancement of the lesions on post contrast MRI, swelling of cauda equina | Diffuse large B-cell lymphoma (DLBCL) | L5-S1 |
5 | Zagami and Granot, 2003 [ ] | Enhancement of the lesions on post contrast MRI, swelling of cauda equina | Diffuse large B-cell lymphoma (DLBCL) | Below cornus |
6 | Tajima et al., 2007 [ ] | Low T2WI, enhancement of the lesions on post contrast MRI, swelling of cauda equina | Diffuse large B-cell lymphoma (DLBCL) | T12-L3 |
7 | Khong et al., 2008 [ ] | Low T2WI, low T1WI, enhancement of the lesions on post contrast MRI, swelling of cauda equina | Diffuse large B-cell lymphoma (DLBCL) | T12-L3 |
8 | Beitzke et al., 2010 [ ] | Diffuse thickening of the cauda equina nerve roots or intradural nodular masses with enhancement of the lesions on post contrast MRI | Diffuse large B-cell lymphoma (DLBCL), massive diffuse interstitial infiltrate of atypical lymphocytic cells (expressed CD20, CD79, and S100) Ki-67 80–90% | Cauda equina nerve roots and the left S1 nerve |
9 | Teo et al., 2012 [ ] | Swelling of cauda equina, slightly high T2WI | Diffuse large B-cell lymphoma (DLBCL) | T12-L3 |
10 | Cugati et al., 2012 [ ] | Swelling of cauda equina, isointense T1WI and T2WI | large B-cell lymphoma | T11-L4 |
11 | Iwasaki et al., 2012 [ ] | Swelling of cauda equina, slightly high T2WI, enhancement of the lesions on post contrast MRI | Diffuse large B-cell lymphoma (DLBCL) | T12-L1 |
12 | Nishida et al., 2012 [ ] | Low T1WI, slightly high T2WI, swelling of cauda equina, enhancement of the lesions on post contrast MRI, increased FDG accumulation | Diffuse large B-cell lymphoma (DLBCL) | T12-L2 |
13 | Nakashima et al., 2014 [ ] | Low T1WI, low T2WI, swelling of cauda equina, enhancement of the lesions on post contrast MRI, increased FDG accumulation | Diffuse large B-cell lymphoma (DLBCL) | T12-S1 |
14 | Broen et al., 2014 [ ] | Low T2WI, swelling of cauda equina, enhancement of the lesions on post contrast MRI, increased FDG accumulation | Diffuse large B-cell lymphoma (DLBCL) | L2-L5 |
15 | Broen et al., 2014 [ ] | Low T2WI, swelling of cauda equina, enhancement of the lesions on post contrast MRI | Diffuse large B-cell lymphoma (DLBCL) | T12-L5 |
16 | Shin et al., 2016 [ ] | Low T2WI, swelling of cauda equina, enhancement of the lesions on post contrast MRI | Diffuse large B-cell lymphoma (DLBCL) | L3-L5 |
17 | Belcastro et al., 2016 [ ] | Swelling of cauda equina, enhancement of the lesions on post contrast MRI, increased FDG accumulation | Diffuse large B-cell lymphoma (DLBCL) | L2-L4 |
18 | Suzuki et al., 2018 [ ] | Enlargement of the cauda equina occupying the dural sac from the L1-S1 level with isointensity to the spinal cord signal on both T1- and T2-weighted imaging; diffuse accumulation of 2-fluoro-2-deoxy-glucose was observed in the cauda equina | Diffuse large B-cell lymphoma (DLBCL), non germinal center (expressed CD20, BCL2, BCL6, and MUM-1) | L4-L5 |
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Lapolla, P.; Maiola, V.; Familiari, P.; Tomei, G.; Gangemi, D.; Ienzi, S.; Arcese, R.; Palmieri, M.; Relucenti, M.; Mingoli, A.; et al. Primary Cauda Equina Lymphoma Mimicking Meningioma. J. Clin. Med. 2024 , 13 , 4959. https://doi.org/10.3390/jcm13164959
Lapolla P, Maiola V, Familiari P, Tomei G, Gangemi D, Ienzi S, Arcese R, Palmieri M, Relucenti M, Mingoli A, et al. Primary Cauda Equina Lymphoma Mimicking Meningioma. Journal of Clinical Medicine . 2024; 13(16):4959. https://doi.org/10.3390/jcm13164959
Lapolla, Pierfrancesco, Vincenza Maiola, Pietro Familiari, Gabriella Tomei, Dominella Gangemi, Sara Ienzi, Roberto Arcese, Mauro Palmieri, Michela Relucenti, Andrea Mingoli, and et al. 2024. "Primary Cauda Equina Lymphoma Mimicking Meningioma" Journal of Clinical Medicine 13, no. 16: 4959. https://doi.org/10.3390/jcm13164959
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Although both Hodgkin and non-Hodgkin lymphoma can occur at any age, they do have a pattern. For non-Hodgkin lymphoma, the risk increases as you get older, with about half the people diagnosed over the age of 65. In Hodgkin lymphoma, cases are predominantly seen in two peaks, often in young adults 20 to 40, and again in older people over 55.
Follicular Lymphoma. The most common presentation is subacute, or chronic asymptomatic peripheral lymphadenopathy, which sometimes persists or waxes and wanes over a period of years. Abdominal, pelvic, or retroperitoneal lymphadenopathy can be bulky without causing gastrointestinal or genitourinary symptoms, and nodal masses are not locally ...
Lymphoma is a group of malignant neoplasms of lymphocytes with more than 90 subtypes. It is traditionally classified broadly as non-Hodgkin or Hodgkin lymphoma. Approximately 82,000 new U.S ...
INTRODUCTION. Hodgkin lymphomas (HL; formerly called Hodgkin's disease) are lymphoid neoplasms in which malignant Hodgkin/Reed-Sternberg (HRS) cells are admixed with a heterogeneous population of non-neoplastic inflammatory cells. HL is divided into two major categories, based on morphology and immunophenotype ( table 1 ):
INTRODUCTION. Non-Hodgkin lymphomas (NHL) comprise a diverse group of hematologic malignancies that are variously derived from B cell progenitors, T cell progenitors, mature B cells, mature T cells, or (rarely) natural killer cells. NHL is seen in patients of all ages, races, and socioeconomic status. Diagnosis and classification of NHL ...
Features of Hodgkin lymphoma include the following: Hodgkin lymphoma (formerly, Hodgkin disease) is a potentially curable lymphoma with distinct histology, biologic behavior, and clinical characteristics. The disease is defined in terms of its microscopic appearance (histology) (see the image below) and the expression of cell surface markers ...
The lymphatic system is part of the body's germ-fighting and disease-fighting immune system. Hodgkin lymphoma begins when healthy cells in the lymphatic system change and grow out of control. The lymphatic system includes lymph nodes. They are found throughout the body. Most lymph nodes are in the abdomen, groin, pelvis, chest, underarms and neck.
Hodgkin lymphoma staging uses the numbers 1 to 4 to indicate the stage. A lower number means the lymphoma cells only involve one or a few areas of lymph nodes. An early-stage cancer is more likely to be cured. As the lymphoma grows to involve more areas of the body, the stage number goes up. A higher number means the cancer is more advanced.
Hodgkin Lymphoma. Characterized by abnormal cells called Reed Sternberg Cells. Reed Sternberg Cells only make up make up 2% of the tumor tissue. Remainder of the tumor is other cells of inflammation. Hodgkin lymphoma was the first to be distinguished from other lymphomas. First to be cured with radiation. First to be cured with chemotherapy.
Other common signs and symptoms of lymphoma include: fever. unexplained weight loss. sweating (often at night) lack of energy. itching. Many of these symptoms may be related to other conditions. For example, it's normal for your lymph nodes to become swollen or enlarged when you're fighting off a viral or bacterial infection.
presentations or involve rapidly progressive adenopathy in more aggressive subtypes. Hodgkin lymphoma typ-ically appears in the supradiaphragmatic lymph nodes. Non-Hodgkin lymphoma can originate ...
Hodgkin lymphoma (HL), formerly called Hodgkin disease, is a rare monoclonal lymphoid neoplasm with high cure rates. Biological and clinical studies have divided this disease entity into two distinct categories: classical Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma (NLP-HL). These two disease entities show differences in the clinical picture and pathology.
Lymphoma. About half of the blood cancers that occur each year are lymphomas, or cancers of the lymphatic system. This system - composed of lymph nodes in your neck, armpits, groin, chest, and abdomen - removes excess fluids from your body and produces immune cells. Abnormal lymphocytes, a type of white blood cell that fights infection, become ...
The clinical manifestations of non-Hodgkin lymphoma (NHL) vary with such factors as the location of the lymphomatous process, the rate of tumor growth, and the function of the organ being compromised or displaced by the malignant process. The Working Formulation classification groups the subtypes of NHL by clinical behavior—that is, low-grade ...
Other typical symptoms of Hodgkin lymphoma include: B symptoms: fever >38°C, drenching night sweats and unintentional weight loss of >10% within the last 6 months. ... The clinical presentation of Hodgkin lymphoma is similar to several other conditions including: ... Hodgkin lymphoma (HL) is a haematological malignancy that arises from B ...
The Clinical Presentation of Lymphomas. From Miguel Islas-Ohlmayer, M.D. Lymphomas, blood cell tumors that usually originate in lymphatic tissues (and can spread to other organs), are among the most diverse and most curable of all malignancies. There are two major groups of lymphomas: About 90 percent are non-Hodgkin lymphomas (NHLs) and about ...
Hodgkin lymphoma has an incidence of 2.8 per 100,000 people per year in the UK, while non-Hodgkin lymphoma has an incidence of 15.5 per 100,000 people per year. ... Presentation. Hodgkin lymphoma commonly presents with painless swollen lymph nodes (lymphadenopathy), often affecting the cervical or supraclavicular nodes in the neck. About 25% of ...
Abdominal pain and distention. Bone pain. Central nervous system (CNS) symptoms. Typical physical examination findings include the following: Pallor (suggesting anemia) Purpura, petechiae, or ecchymoses (suggesting thrombocytopenia) Findings in patients with an advanced high-grade lymphoma may include the following: High fever.
The variable clinical presentation of lymphoma is a challenge for the ENT specialist. Fast diagnosis is crucial for rapid treatment, especially in highly aggressive NHL like the Burkitt-lymphoma and HL. ... Typical symptoms can include an indolent lymphadenopathy (here, we concentrate on the cervical lymph nodes), fatigue, occasionally B ...
Many types of treatments exist for lymphoma. Treatments include radiation, chemotherapy, immunotherapy, targeted therapy and bone marrow transplant, also called stem cell transplant. Sometimes, a combination of treatments is used. The treatment that's best for you will depend on the kind of lymphoma that you have.
Non-Hodgkin's lymphoma was 4 times more common than Hodgkin's disease. The vast clinical spectrum of lymphoma sometimes delays its diagnosis, leading to its eventual presentation in late stages. A general awareness is hence required among the health professionals regarding its varied clinical presen …
B-cell lymphoblastic lymphoma (B-LBL) is a subtype of non-Hodgkin lymphoma characterized by the proliferation of abnormal B-cell lymphoblasts in lymphoid tissues. Typical presentations include lymphadenopathy, mediastinal mass, and involvement of organs such as the liver and spleen, but extranodal sites can also be affected. A previously healthy 20-month-old male child presented to the ...
Background: Spinal cord lymphomas represent a minority of extranodal lymphomas and often pose diagnostic challenges by imitating primary spinal tumors or inflammatory/infective lesions. This paper presents a unique case of primary cauda equina lymphoma (PCEL) and conducts a comprehensive review to delineate the clinical and radiological characteristics of this rare entity. Case Report: A 74 ...