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Non-Hodgkin Lymphoma

This information should not be used for self-diagnosis or in place of a qualified physician's care.

Revised Nov 2020

The basics
  • Guidelines for treating this cancer have been developed by the Lymphoma Tumour Group.
  • For health professional information on treating this cancer, please see our Cancer Management Guidelines.
  • These cancers are also called NHL or malignant lymphoma, or lymphoid cancers. Dr. Thomas Hodgkin first described lymphoma in the 1830s.
  • There are many types of lymphoma with different names. Waldenstrom's Macroglobulemia, lymphomatoid granulomatosis, lymphomatoid papulosis, and MALT are all types of lymphoma. Most other types have "lymphoma" in the name.
  • Hodgkin lymphoma has a separate page of information. Mycosis fungoides, cutaneous T-cell lymphoma, and Sezary syndrome are discussed in Skin lymphoma.
  • Lymphomas are cancers that arise from lymphocytes, which are white blood cells that circulate primarily through the lymphoid system. The lymphoid (or lymphatic) system is part of the immune system, which protects against infection.
  • The lymphoid system consists of: lymph nodes, lymphatic vessels, lymph fluid and lymph tissue in other organs such as the spleen, thymus, bone marrow.
  • The lymph nodes are small organs occurring in many places in the body, particularly in the chest, neck, armpit, groin and amongst the blood vessels of the intestines. Lymphatic vessels are a network to carry lymph fluid between the nodes and through the body.
  • Non-Hodgkin lymphomas are called B-cell or T-cell lymphoma depending on the type of lymphocyte that has become cancerous.
    • B-cell lymphocytes come from the bone marrow. 90% of lymphomas are B-cell.
    • T-cell lymphocytes come from the thymus, a gland in the central part of the chest. T-cell lymphomas are less common and have a greater chance of recurring.
  • Abnormal or cancerous lymphocytes (T-cell or B-cell) may:
    • stay in the lymph nodes
    • form solid tumours in the body
    • rarely, like leukemia, circulate in the blood
  • Non-Hodgkin lymphomas (see link) can show up almost anywhere in the body. The gastrointestinal tract (GI) is a common site, as are the liver, spleen or bone marrow. Lymphomas can involve the coverings of the brain (the meninges) and the fluid surrounding the brain (cerebrospinal fluid or CSF). The eyes, testicles or sinuses are other areas of the body where lymphoma may grow.
What causes it and who gets it?

Listed below are some of the known risk factors for this cancer. Not all of the risk factors below may cause this cancer, but they may be contributing factors.

  • Previous exposure to radiation or chemotherapy can make people vulnerable to lymphoma.
  • Exposure to some chemicals, including herbicides, may increase risk.
  • People who take drugs that suppress the immune system have a higher risk of lymphoma, e.g. people who have had organ transplants.
  • Some diseases or infections are risk factors; eg. HIV, Epstein-Barr, H. pylori (a gastrointestinal bacteria), HTLV-1 (human T-lymphotropic virus) or hepatitis C.
  • Risk increases with age.
  • Researchers are looking at other possible risk factors, such as obesity, autoimmune diseases, or familial factors.
  • Changes in research and treatments for lymphoma are ongoing and frequent. The death rate from non-Hodgkin lymphoma in Canada decreased by over 3% per year every year from 2001 - 2006.
  • Statistics
    • BC
    • Canada
      NOTE:  Available statistics do not have information about the inclusion of transgender and gender diverse participants. Unless specified, it is unknown how these statistics apply to transgender and gender diverse people.  Patients are advised to speak with their primary care provider or specialists about their individual considerations and recommendations.
Can I help to prevent it?

No. Since the causes are not understood, there is no known way of preventing this disease.

Screening for this cancer

No effective screening program exists for this cancer yet.

Signs and symptoms
Most symptoms of lymphoma are also found in other less serious disorders. These symptoms are usually caused by something else, and are not likely to be a sign of lymphoma. However, it is always important to have symptoms checked by a health professional. 

Symptoms include:
 
  • Painless enlarged lymph nodes in neck, groin or armpit
  • Fatigue
  • Shortness of breath
  • Itchiness on the trunk of the body
  • Unusual back or abdominal pain
  • Abdominal swelling
The following are called "B symptoms" and they are important in planning treatments:

  • Persistent fever whose cause is unknown
  • Unexplained night sweats, sometimes so much that the bed sheets have to be changed
  • Unexplained weight loss
Diagnosis & staging

Diagnosis

These are tests that may be used to diagnose this type of cancer.

  • An accurate diagnosis in lymphoma is very important since specific types of lymphoma respond to very different forms of treatment, making them different from other cancers.
  • A very thorough medical and physical examination will be done, with attention to lymph nodes, spleen, liver and pain in bones.
  • Blood and urine samples will be taken for laboratory testing, including a complete blood count, blood chemistry tests, and cell and tissue analyses.
  • A chest X-ray will be taken.
  • CT scans will be done, of the chest, abdomen and/or pelvis, to look for signs of cancer in those organs and nearby lymph nodes.
  • A surgical biopsy removes the affected lymph node(s) or other tissue, and specialists will look at the cells under a microscope to find out what kind of lymphoma it is, and where it is located.
  • Additional tests are sometimes done, such as:
    • Imaging scans of bones or organs such as the liver and spleen
    • Gastrointestinal tests including endoscopy, which is using a scope to view the stomach and intestines
    • Spinal tap (lumbar puncture) to examine the cerebrospinal fluid (CSF).
  • Non-Hodgkin lymphomas do not necessarily spread in a regular pattern, unlike Hodgkin lymphoma which is usually somewhat predictable and spreads to adjacent lymph nodes.

For more information on tests used to diagnose cancer, see our Recommended Websites, Diagnostic Tests section.

Types and Stages
  • Subtyping of lymphoma is based on the following characteristics:
    • Whether the pattern is follicular (cells clumped in the lymph node) or diffuse (spread out).
    • Whether the size or types of cells affected are large, small or a mixture of both.
    • How well-differentiated (mature) the cells are.
Indolent (slow growing)

B-cell

  • Small lymphocytic (which is very similar to CLL chronic lymphocytic leukemia and is treated in the same way)
  • Lymphoplasmacytic, including Waldenstrom’s Macroglobulinemia
  • Follicular, grade 1, 2 or 3 A
  • Marginal zone:
    • MALT (Mucosa-associated lymphoid tissue)
    • nodal
    • splenic
T-cell
  • Mycoses Fungoides
Aggressive (faster growing)
B-cell
  • Follicular, grade 3 B
  • Mantle cell
  • Diffuse large cell, any type (including primary mediastinal, T-cell rich B-cell, immunoblastic and intravascular variants)
  • Burkitt-like (small noncleaved cell)
T-cell
  • Peripheral T cell, Angioimmunoblastic (AIL)
  • Nasal T/NK cell
  • Subcutaneous panniculitic
  • Enteropathy associated
  • Anaplastic large cell (CD30 positive) including null cell
Special

B-cell

  • Burkitt

T-cell

  • Lymphoblastic

Lymph tissue is also studied with molecular marker studies and genetic studies, to help with treatment planning. 

Stages

Staging describes the extent of a cancer. In general a lower number in each category means a better prognosis. The stage of the cancer is used to plan the treatment.

  • Non-Hodgkin's lymphomas are divided into groups; low grade or indolent lymphomas which tend to grow slowly; and higher grade or aggressive lymphomas which tend to grow faster.
  • The grades of lymphoma can be further classified into approximately twenty different subtypes, based on an international classification scheme called the World Health Organization Classification. The types are identified by examining lymphatic tissue with a microscope and in the laboratory.

The type of staging that BC Cancer uses is based on the Ann Arbor classification, plus we consider the size of the individual tumour(s).

Stage I 
Involves a single lymph node region 

Stage II 
Involvement of two or more lymph node regions on the same side of the diaphragm*  

Stage III 
Involvement of lymph node regions on both sides of the diaphragm*  

Stage IV 
Extensive spread of the disease outside the lymph system, into other organs or kinds of tissue. 

* The diaphragm is a large muscle below the ribs, which divides the body into top and bottom, separating the chest area from the abdomen.

  • Each stage is divided into A and B categories. The letter is added to the stage number, for example Stage IIB or Stage IIIA.
    • "A" patients have no specific symptoms from the "B" list.
    • "B" symptoms are unexplained weight loss of more than 10% in the six months before diagnosis, or unexplained fevers above 38 C (100.4 F), or persistent heavy night sweats.
Treatment

Treatment

Cancer therapies can be highly individualized – your treatment may differ from what is described below. 

Virtually all lymphoma patients can be helped with proper treatment. Approximately 50% can be cured and 50% can have their disease made better for periods of time varying from several months to many years. 

New treatments or clinical trials may be offered to patients, other than ones described here. 

Treatment plans are different for lymphomas with different grade, type, and stage. The patient's age, health, and other concerns are also part of planning treatment for lymphoma. 

Lymphoma in some situations or areas of the body needs special consideration, for example:
  • Lymphoma related to a previous or existing infection, such as AIDS, HTLV-1, or hepatitis-B or C.
  • Lymphoma appearing in certain parts of the body: GI tract, testes, central nervous system (brain, spinal cord), eye, paranasal sinus, or skin.
  • Transplant related lymphomas
  • Discordant histology lymphomas, which have more than one kind of lymphoma appearing at the same time.
In these cases, extra tests may be done and treatment may be different than described below. 

Observation
For some slow growing, indolent lymphomas, no treatment is needed right away. 

Chemotherapy
  • Using a combination of different drugs, along with radiation therapy, has produced excellent results for many subgroups of non-Hodgkin's lymphoma.
  • Many different chemotherapy drugs are used to treat lymphoma.
  • Immunotherapy drugs called monoclonal antibodies are often used. This is sometimes called biological therapy.
  • The side-effects of the chemotherapy protocols (treatment plans) for lymphoma are quite variable. Some protocols do not cause any side-effects. Most protocols cause moderate side-effects and a few protocols cause major side-effects. The side-effects associated with each treatment plan are specific to the drugs and schedule being used and should be discussed with your oncologist.
Radiotherapy
  • Early stage follicular lymphomas are usually treated with radiation therapy and have excellent results.
  • Stages I and II diffuse lymphomas are usually treated with radiation and brief chemotherapy.
  • Radiation treatment may be added after chemotherapy, based on the results of diagnostic scans such as CT and PET following treatment.
Bone Marrow Transplant
  • Extremely high dose chemotherapy and bone marrow transplantation can be effective if regular dose chemotherapy fails. If you will be undergoing a bone marrow transplant as part of your treatment, you may be interested in  the information from the Leukemia/BMT Program of BC.

Follow-up after treatment

  • Guidelines for follow-up after treatment are covered on our website. Follow the link and use the left side menu to jump to the Follow Up section.
  • You will be returned to the care of your family doctor or specialist for regular follow-up. If you do not have a family physician, please discuss this with your BC Cancer oncologist or nurse.
  • Follow-up testing is based on your type of cancer and your individual circumstances.
  • Life after Cancer focuses on the issues that cancer survivors can face.
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SOURCE: Non-Hodgkin Lymphoma ( )
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