Non-Hodgkin’s Lymphoma

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Less Common Non-Hodgkin’s Lymphoma B-Cell Subtypes

Most people with B-cell non-Hodgkin’s lymphoma (NHL) have diffuse large B-cell lymphoma or follicular lymphoma. Less common subtypes of B-cell non-Hodgkin’s lymphoma include:

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 and are usually diagnosed in adults over age 50.

Approximately 15,000 new cases of CLL and 5,000 new cases of SLL are diagnosed annually in the United States. SLL accounts for about 5 to 10 percent of all lymphomas.

In addition to the typical NHL symptoms, people with CLL or SLL may also experience 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. Treatments used for CLL and SLL include chemotherapy and monoclonal antibodies that recognize specific proteins on the surface of lymphoma cells and destroy the cells. Patients with CLL that does not respond to therapy or with disease that transforms into a more aggressive (fast-growing) form may be candidates for stem cell transplant.

Mantle Cell Lymphoma

Mantle cell lymphoma (MCL), which accounts for about 5 percent of lymphomas, 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 indolent (slow growing) 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. In some cases, stem cell transplant may be an option. There are also a number of treatments being investigated in clinical studies.

Marginal Zone B-Cell Lymphoma

Marginal zone lymphomas, which are slow growing, account for about 5 to 10 percent of all lymphomas. This disease is typically diagnosed in patients who are in their 60s. There are three main subtypes of marginal zone lymphomas.

Extranodal Marginal Zone B-Cell Lymphoma (Mucosa-Associated Lymphoid Tissue Lymphoma)

These lymphomas start outside of the lymph nodes and are the most common subtype of marginal zone lymphomas, accounting for nearly 10 percent of all B-cell lymphomas.

There are two subtypes of mucosa-associated lymphoid tissue (MALT) lymphomas:

  • Gastric MALT, which develops in the stomach
  • Non-gastric MALT, which develops in the lungs, skin, thyroid, salivary glands, and tissues surrounding the eye

Usually the disease is confined to the area where it begins (localized). In many cases, people with MALT have a history of inflammation, bacterial or viral infections, or autoimmune disorders. Their lymphoma is often linked to bacterial or viral infections.

For gastric MALT, antibiotics are typically the first treatment since treating the infection often cures the lymphoma. Patients who relapse may be treated with immunotherapy, chemotherapy, radiation, or surgery. Treatment for non-gastric MALT is based on the area where the disease is found in the body; it usually consists of local surgery or radiation therapy.

Nodal Marginal Zone B-Cell Lymphoma

This disease, found mainly in women, accounts for about 2 percent of all B-cell lymphomas. It usually stays in the lymph nodes, although lymphoma cells may also be found in the bone marrow.

Often treatment for this disease begins with watchful waiting, but when treatment is indicated, 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, accounting for only about 1 percent of all B-cell lymphomas. It is typically found in men and has been associated with hepatitis C.

Treatment may include watchful waiting until or unless symptoms become problematic. Treatment options include surgery to remove the spleen, low-dose radiation of the spleen, and immunotherapy with or without chemotherapy.

Burkitt Lymphoma

Burkitt lymphoma, named for the doctor who first described the disease, accounts for about 2 percent of all lymphomas. This is a very aggressive lymphoma found mostly young men, with 30 being the average age.

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 and can spread to the brain and spinal fluid. Patients are usually treated with combination chemotherapy, and more than half are cured.

Hairy Cell Leukemia

Although it is called leukemia, this is cancer of lymphocytes (a type of white blood cell) in the bone marrow, spleen, and blood, and it is sometimes considered a type of lymphoma. This slow-growing disease gets its name from the way the cells look under the microscope: They have fine projections on their surface that make them look hairy. Hairy cell leukemia (HCL) is extremely rare, with fewer than 1,000 people diagnosed in the United States each year. Men are more likely to get HCL than women, and the average age is around 55.

Treatment may not be needed for patients with early-stage HCL. 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 from most lymphomas and may include headaches, confusion, vision problems, facial paralysis, and seizures.

Patients may initially be treated with corticosteroids to control symptoms and reduce swelling. Other treatments include single-agent or combination chemotherapy, radiation therapy, and occasionally stem cell transplant.

Lymphomatoid Granulomatosis

This is a rare disease caused by overproduction of lymphocytes mainly in the lungs. It may also affect the skin, kidneys, liver, or central nervous system. Lymphomatoid granulomatosis affects twice as many men as women and is usually diagnosed in people between the ages of 30 and 50. It is related to immunodeficiency, especially Epstein-Barr virus, and is often diagnosed together with different types of large B-cell lymphoma.

Due to its association with immunodeficiency, it may be treated with antiviral drugs, as well as steroids and chemotherapy. Approximately half of patients respond to treatment. Patients whose disease progresses or who do not respond to therapy are typically treated with combination chemotherapy.