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 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.
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Resources
Cases in Lymphoma - Marginal Zone Lymphoma
Paper: Marginal zone-related neoplasms of splenic and nodal origin, Arcaini L et al., Haematologica/journal of hematology vol. 88(01):January 2003 (this paper is technical but extensive with a long list of references)
Oncologychannel - Non-Hodgkin's Lymphoma
Lymphomation - Marginal Zone Lymphomas
The Hematopathology of Lymphoma
Abstract: Splenic Marginal Zone Lymphoma, Franco V et al. Blood. 2003 Apr 1; 101(7): 2464-72. Epub 2002 Nov 21
Abstract: 7q31-32 Allelic Loss Is a Frequent Finding in Splenic Marginal Zone Lymphoma Mateo, M et al. American Journal of Pathology. 1999;154:1583-1589
del(7q) in non-Hodgkin's Lymphoma (NHL) - Atlas of Genetics and Cytogenetics in Oncology and Haematology (France)
Information from a pathology update in the Cancer Control Journal: Indolent Lymphomas
Related Articles
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:
Childhood Non-Hodgkin's Lymphoma Information Pages
The Lymphoma Information Network
Books
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
