In the United States, about 90 percent of non-Hodgkin lymphomas (NHLs) begin in the B cells. There are several subtypes of B-cell NHL. The most common are:
Together these two subtypes make up about half of all cases of non-Hodgkin’s lymphoma.
Less common subtypes of B-cell NHL include:
- Chronic lymphocytic leukemia/small lymphocytic lymphoma
- Mantle cell lymphoma
- Marginal zone B-cell lymphoma
- Burkitt lymphoma
- Waldenström macroglobulinemia (lymphoplasmacytic lymphoma)
- Hairy cell leukemia
- Primary central nervous system lymphoma
- Lymphomatoid granulomatosis
- Cutaneous lymphoma, B-cell type
Diffuse large B-cell lymphoma (DLBCL) is a fast-growing (aggressive) lymphoma. This is the most common subtype, accounting for about 30 percent of NHLs in this country.
- About one-third of these lymphomas are confined to one part of the body (localized) at diagnosis.
- Because DLBCL advances very quickly, it is usually treated right away with combination chemotherapy and the monoclonal antibody rituximab (Rituxan).
- After initial treatment, most patients experience long-term remission or are cured.
- Patients whose disease relapses or does not respond to treatment (refractory disease) may have high-dose chemotherapy and a bone marrow transplant.
Different subtypes of DLBCL have different outlooks and responses to treatment.
- Primary mediastinal B-cell lymphoma is typically confined to the area where it begins — in the chest in the space between the lungs (mediastinum).
- Intravascular large B-cell lymphoma is a rare, aggressive subtype that is typically throughout the body by the time it is found.
- Primary effusion lymphoma is a rare, aggressive subtype associated with human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS). It may be treated with combination chemotherapy and, for patients who are HIV-positive, antiretroviral therapy.
Follicular lymphoma is often slow growing (indolent). Follicular means the cells tend to grow in a circular pattern. About 20 percent of NHLs are follicular lymphoma.
- This subtype occurs in many lymph nodes throughout the body as well as in the bone marrow.
- Since follicular lymphoma typically grows slowly, you and your doctor may decide to monitor the disease rather than treat it. This is known as watchful waiting.
- Treatments include single-agent or combination chemotherapy, radiation or immunotherapy, which uses the body’s immune system to fight cancer.
- Many patients have long-term remission after treatment, but in most the disease relapses or doesn’t respond to treatment. These patients may have high-dose chemotherapy and a bone marrow transplant or treatments being tested in clinical studies.
- Over time, about 30 percent of follicular lymphomas transform into aggressive diffuse B-cell lymphoma.
Chronic lymphocytic leukemia/small lymphocytic lymphoma
The only difference between these closely related diseases is the location of the cancer cells.
- In chronic lymphocytic leukemia (CLL), the leukemia version, the cancer cells are primarily in the blood and bone marrow.
- In small lymphocytic lymphoma (SLL), the lymphoma version, the cancer cells are mainly in the lymph nodes and spleen.
Both CLL and SLL are slow-growing diseases.
- Along with typical NHL symptoms, people with CLL or SLL may have shortness of breath, anemia, bruising and frequent infections.
- Since CLL and SLL are often slow growing, you and your doctor may decide to monitor the disease rather than treat it. This is called watchful waiting.
- Treatments include chemotherapy and monoclonal antibodies that recognize specific proteins on the surface of lymphoma cells and destroy the cells.
- If the disease does not respond to therapy or it transforms into a more fast-growing (aggressive) form, doctors may recommend a bone marrow transplant.
Mantle cell lymphoma
Mantle cell lymphoma (MCL) typically affects men between the ages of 50 and 70. It has often spread throughout the body by the time it is diagnosed and may be in the lymph nodes, bone marrow, blood, gastrointestinal tract and spleen.
- MCL can be difficult to classify as slow growing (indolent) or aggressive, but it is often treated as an aggressive type because it is hard to treat.
- Treatment may include combining chemotherapy and immunotherapy, which uses the body’s immune system to fight cancer.
- Bone marrow transplant may be an option for some people.
- Many treatments are being investigated in clinical studies.
Marginal zone B-cell lymphoma
Marginal zone lymphomas, which are slow growing, are typically diagnosed in people in their 60s. There are three main subtypes.
Extranodal marginal zone B-cell lymphoma (mucosa-associated lymphoid tissue lymphoma, MALT)
These lymphomas start outside the lymph nodes. Usually the disease is confined to the area where it begins. In many cases, patients have a history of inflammation, bacterial or viral infections or autoimmune disorders. Their lymphoma is often linked to infections.
There are two subtypes:
- Gastric MALT, which develops in the stomach. Treating the infection often cures the lymphoma, so antibiotics are typically the first treatment. If the disease relapses, doctors may recommend immunotherapy, chemotherapy, radiation or surgery.
- Non-gastric MALT, which develops in the lungs, skin, thyroid, salivary glands and tissues surrounding the eye. Treatment depends on where the disease is found. Usually treatment consists of surgery or radiation therapy.
Nodal marginal zone B-cell lymphoma
This disease, found mainly in women, usually stays in the lymph nodes, although lymphoma cells may also be found in the bone marrow. Often treatment begins with watchful waiting. Options include radiation therapy and chemotherapy.
Splenic marginal zone B-cell lymphoma
This lymphoma, found in the bone marrow, blood and spleen, is very rare. It is typically found in men and has been associated with hepatitis C. Treatment may include watchful waiting unless symptoms become a problem. Treatment options include surgery to remove the spleen, low-dose radiation of the spleen and immunotherapy with or without chemotherapy.
Burkitt lymphoma is a very aggressive lymphoma found mostly in young men. The average age is 30. In the United States, the disease usually starts as a large tumor in the abdomen. It can also start in the testicles, ovaries or other organs. Patients are usually treated with combination chemotherapy, and more than half are cured.
Hairy cell leukemia
Although it is called leukemia, this slow-growing cancer of lymphocytes (a type of white blood cell) is sometimes considered lymphoma. It gets its name from the way the cells look under the microscope: They have fine projections on their surface that make them look hairy. The disease is extremely rare, with fewer than 1,000 people diagnosed in the United States each year.
Treatment may not be needed in the early stages. Some patients need an occasional blood transfusion. If the spleen becomes enlarged or blood counts drop, treatment may include surgery to remove the spleen and chemotherapy.
Primary central nervous system lymphoma
This rare lymphoma is more common in people with immune system diseases, such as HIV/AIDS. It often involves the brain (primary brain lymphoma) but may also be found in and around the spinal cord and eyes. Lymphoma of the eye is called primary intraocular lymphoma.
Eventually the disease spreads throughout the central nervous system. The symptoms are different than for most lymphomas and may include headaches, confusion, vision problems, facial paralysis and seizures.
To start, patients may receive corticosteroids to control symptoms and reduce swelling. Other treatments include chemotherapy, radiation therapy and occasionally bone marrow transplant.