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Splenic Marginal Zone Lymphoma

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Splenic marginal zone lymphoma (SMZL) is an indolent B-cell lymphoma confined to the spleen. The spleen is an organ in the chest important for fighting infection.  It is a rare lymphoma, accounting for <1% of all non-Hodgkin's lymphomas.  This lymphoma occurs in adults and is slightly more frequent in women than in men.  Other forms of marginal zone lymphoma are covered in a separate page.

Presentation

The appearance of symptoms related to the disease often occurs several years after the first biological manifestation.  The cancer is marked by massive splenomegaly (enlargement of the spleen) and peripheral blood and bone marrow involvement, usually 
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without adenopathy (swollen lymph nodes).

Causes

It has been suggested that the deletion of the 7q31-32 chromosome may be specific to this lymphoma, indicating that the normal gene in this region is used to suppress tumors.  Other cytogenetic alterations include abnormalities in chromosomes 1, 7, and 8.

Treatment

Therapeutic options include treatment abstention, splenectomy (removal of the spleen), splenic irradiation, and chemotherapy.  Splenectomy may result in a prolonged remission.  

Since this is a B-cell lymphoma, it is possible that treatment with recent monoclonal antibody drugs (rituxan, bexxar, etc.) may be effective.

Given the rarity of this form of lymphoma along with recent classification of SMZL as a distinct NHL entity, you should consult your doctor who will search current literature for the best treatment options.

Further Reading / In-Depth

Non-Hodgkin's Lymphomas, Peter M. Mauch (Editor), James O. Armitage (Editor), et al., 2004.

  • Section IV: Pathology, Biology, Clinical Evaluation, and Treatment Section
    • Chapter 16: Splenic Marginal Zone Lymphoma

Web Resources:


Information Sources


Non-Hodgkin's Lymphoma Information Pages:

    Non-Hodgkin's Lymphomas
    Non-Hodgkin's Lymphoma: Diagnosis
    Non-Hodgkin's Lymphoma: Aggressive Lymphomas
    Non-Hodgkin's Lymphoma: Indolent Lymphomas
    Non-Hodgkin's Lymphoma: Treatment
    Non-Hodgkin's Lymphoma: Resources
    Bone Marrow and Stem Cell Transplants

Other Pages of Interest:


This page is a work in progress - if you have more complete information, references, or other information please contact the author. The author is not in the medical field and does not warrant the correctness of the material on this page or the sites linked - please take online information and consult with your own medical team to make informed decisions.

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Updated October 30, 2005