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Splenic marginal zone lymphoma

Splenic marginal zone lymphoma
Classification and external resources
Specialty Hematology and oncology
ICD-9-CM 200.3
ICD-O M9689/3
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Antigen Status
CD20 Positive
CD79a Positive
CD5 Negative
CD10 Negative
CD23 Negative
CD43 Negative
cyclin D1 Negative

Splenic marginal zone lymphoma (SMZL) is a lymphoma made up of B-cells that replace the normal architecture of the white pulp of the spleen. The neoplastic cells are both small lymphocytes and larger, transformed blasts, and they invade the mantle zone of splenic follicles and erode the marginal zone, ultimately invading the red pulp of the spleen. Frequently, the bone marrow and splenic hilar lymph nodes are involved along with the peripheral blood. The neoplastic cells circulating in the peripheral blood are termed villous lymphocytes due to their characteristic appearance.

Under older classification systems, the following names were used:

The cell of origin is postulated to be a post-germinal center B-cell with an unknown degree of differentiation.

With splenic involvement a requirement for a diagnosis of SMZL, splenomegaly is seen in almost all patients, commonly without lymphadenopathy. Aside from the uniform involvement of the spleen, the bone marrow is frequently positive in patients with SMZL. Nodal and extranodal involvement are rare.

Circulating lymphoma cells are sometimes present in peripheral blood, and they occasionally show short villi at the poles of cells and plasmacytoid differentiation.

Autoimmune thrombocytopenia and anemia sometimes seen in patients with SMZL. Circulating villous lymphocytes are sometimes observed in peripheral blood samples. A monoclonal paraprotein is detected in a third of patients without hypergammaglobulinemia or hyperviscosity.


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