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Non-Hodgkin lymphoma

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Splenic marginal zone lymphoma

Marginal zone lymphomas are B-cell lymphomas. They are not very common and account for a small percentage of non-Hodgkin lymphomas (NHL). There are 3 main types of marginal zone lymphomas:

Splenic marginal zone lymphoma (SMZL) is a rare type of NHL. It accounts for less than 2% of all cases of NHL. It may also be called splenic lymphoma with villous lymphocytes. SMZL most often affects the spleen, bone marrow and blood, often without any lymph node involvement. People with SMZL often have an enlarged spleen (splenomegaly), anemiaanemiaA reduction in the number of healthy red blood cells. and low platelet counts (thrombocytopenia). The liver may also be enlarged (hepatomegaly).

SMZL occurs most often in the elderly.

SMZL is a slow-growing (indolent) lymphoma. It can change (transform) into an aggressive large B-cell lymphoma, but this is not very common.


There are several treatment options for SMZL.

Watchful waiting

Because SMZL is a slow-growing (indolent) lymphoma, people may not need treatment right away unless they have symptoms.


People with SMZL often respond very well when their spleen is removed with surgery (splenectomy).

Radiation therapy

When a splenectomy is not an option, external beam radiation therapy to the spleen may be used to treat SMZL.


SMZL doesn’t respond as well to chemotherapy as some of the other indolent lymphomas, but chemotherapy may still be used. Chemotherapy drugs that may be used to treat SMZL include:

  • fludarabine (Fludara)
  • cladribine (2-CDA, Leustatin)
  • pentostatin (deoxycoformycin, Nipent)

These drugs seem to be more effective than other types of chemotherapy drugs like cyclophosphamide (Cytoxan, Procytox) or chlorambucil (Leukeran). Combinations of chemotherapy drugs may also be used, such as CVP – cyclophosphamide, vincristine (Oncovin) and prednisone in combination with rituximab (Rituxan).

Biological therapy

Monoclonal antibodies are a type of biological therapy that is effective in treating certain types of NHL. Rituximab (Rituxan) may be used alone or in combination with chemotherapy. It seems to improve the outcome for people with SMZL.

A very small number of people with SMZL will also have hepatitis C infection (HCV). Interferon alfa (Intron A, Wellferon) may be used alone or in combination with a drug called ribavirin, which is used to treat viral infections like HCV. This combination has been shown to be helpful.

For more detailed information on specific drugs, go to sources of drug information.


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