Indolent non-Hodgkin lymphoma (NHL) subtypes progress slowly. They make up about 30 percent of all NHL cases in the United States. Indolent subtypes include:
- Cutaneous T-cell lymphoma (mycosis fongoides and Sézary syndrome)
- Follicular lymphoma
- Lymphoplastic lymphoma and Waldenström macroglobulinemia
- Marginal zone lymphoma
- Small cell lymphocytic lymphoma (SLL) and chronic lymphocytic leukemia (CLL)
Treatment for indolent NHL ranges from a watch-and-wait approach to aggressive therapy. Indolent NHL management or treatment is highly individual. Your doctor considers a number of factors such as:
- Your outlook for recovery
- The disease's stage
- Your overall health
If you don't have symptoms and your disease isn't progressing, your doctor may recommend the watch-and-wait approach. You'll get no immediate therapy, but your doctor will monitor you closely for any changes. But having an indolent NHL doesn't automatically qualify you for watch and wait: Some types of indolent NHL need aggressive initial therapy.
Drugs Used for Indolent Non-Hodgkin Lymphoma
If you need treatment right away, you'll typically undergo chemotherapy. Doctors commonly combine up to five chemotherapy drugs to treat indolent NHL. Each drug type works in a different way to kill the cancerous cells. Combining drug types can strengthen their effectiveness.
A common chemotherapy regimen is a group of drugs known as CVP. (The names of the combinations are usually abbreviations for the first letter of the drugs used.) CVP combines the following drugs:
- Cyclophosphamide (Cytoxan ®)
- Vincristine (Oncovin ®)
For certain subtypes, doctors typically add the monoclonal antibody rituximab (Rituxan®) to this mix, either combined with CVP or given later, which can aid in the treatment's effectiveness.
Treating Specific Indolent Subtypes
Cutaneous T-cell lymphoma usually starts in a T cell in the skin. It can become more advanced if it spreads to other organs. Two common types of cutaneous T-cell lymphoma are mycosis fungoides and Sézary syndrome. This NHL subtype can be difficult to diagnose in its early phases. Treatment depends on whether it's still present in the lymph nodes and the severity of skin lesions. It may include topical chemotherapy drugs that you directly apply to affected skin combined with ultraviolet light therapy and electron beam therapy. The drug commonly used with ultraviolet light therapy is psoralen. This treatment combination is abbreviated and referred to as PUVA. If the disease is widespread, your doctor may use single- or multidrug chemotherapy or extracorporeal photopheresis, a method of removing blood from your body and treating it with ultraviolet light before returning it to your body.
To read more about cutaneous T-cell lymphoma and treatment options, download or order The Leukemia & Lymphoma Society's (LLS's) free fact sheet Cutaneous T-Cell Lymphoma Facts.
Follicular lymphoma (FL) is the second most common subtype of non-Hodgkin lymphoma (NHL), making up about 20 percent of all NHL cases. The lymphoma starts in cells where a translocation between chromosomes 14 and 18 takes place. This causes excess production (overexpression) of the gene BCL-2, which can make the cells resistant to therapy. However, treatment can keep the indolent disease in check for many years.
FL has a high risk of transforming into an aggressive disease. FL can be treated with:
- Radiation therapy
- Chemotherapy followed by radiation therapy
- A watch-and-wait approach
- A radioactive monoclonal antibody
- Stem cell transplantation
Taking part in a clinical trial can also be a good treatment option.
Doctors have created a scoring system called the Follicular Lymphoma International Prognostic Index that can help them predict which patients have a higher risk of the disease returning (recurrence). Doctors design a treatment plan around the patients' score. Factors that raise risk for FL patients include:
- Older than 60
- Stage III or IV disease
- More than four lymph nodes involved
- An elevated level of lactic dehydrogenase (a type of protein) in the blood
- A low (less than 12 grams per deciliter) hemoglobin concentration in the blood
- Treatment options for transformed FL are described in Treatment for Aggressive NHL Subtypes.
Lymphoplasmacytic lymphoma and Waldenström macroglobulinemia are two closely related NHL subtypes that start in a B cell. Both can become more advanced if the lymphoma spreads to other organs such as the lungs and digestive tract. Your doctor may suggest a watch-and-wait approach if the disease is in its early stages. Otherwise, you may be treated with therapy regimens that include a combination of biological agents (treatment that stimulates the immune system to fight cancer), signaling inhibitors (drugs that block progrowth and survival signals), and chemotherapy. Recently, ibrutinib (Imbruvica®) became the first ever drug approved for the treatment of symptomatic patients with WM.
A serious complication of both diseases is called hyperviscosity syndrome, in which the blood thickens. This leads to inadequate blood flow, causing such symptoms as headache, blurred vision and mental confusion. To treat the syndrome, your doctor may have you undergo plasmapheresis, a method of removing from your blood excess antibodies that cause the disorder. This is followed by treatment to control the lymphoma.
For more about Waldenström macroglobulinemia and treatment options, download or order LLS's free fact sheet Waldenström Macroglobulinemia Facts.
Marginal zone lymphoma can develop in either the lymph nodes (nodal) or elsewhere in the body (extranodal) but usually doesn't spread. Nodal marginal zone B-cell lymphoma (also called monocytoid B-cell lymphoma) is a rare form of the disease that's found in the spleen and blood.
Mucosa-associated lymphoid tissue (MALT) lymphoma is a more common type that develops outside the lymph nodes, including the digestive (gastrointestinal) tract, eyes, thyroid gland, salivary glands, lungs and skin. MALT has been associated with a history of autoimmune disease and bacteria infection, including Helicobacter pylori (H. pylori). Doctors treat MALT with potent combinations of antibiotics, resulting in cures for some patients without having to undergo radiation therapy or chemotherapy.
Small cell lymphocytic lymphoma (SLL) and chronic lymphocytic leukemia (CLL) are similar NHL subtypes. They tend to affect the same age groups (median age of patients is 65 years), have common signs and symptoms and be slow-growing. The differences lie in where they develop: SLL mostly affects the lymph nodes and lymphoid tissue; CLL mostly affects blood and bone marrow but can spread to the lymph nodes. Treatment for both is the same: chemotherapy with the drug bendamustine (Treanda®), which is used to treat CLL. It's proven to be effective in SLL patients in clinical trials after treatment with rituximab (Rituxan®) either alone or with other drugs.
To read more about CLL and its treatment options, download or order LLS's free booklet Chronic Lymphocytic Leukemia.