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SMZL : Clinical Features

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HemePath_Spleen_MZL15.jpg

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Splenic Marginal Zone Lymphoma (SMZL) - Clinical Features: SMZL is an indolent neoplasm that makes up 1-2% of all lymphomas. It affects older individuals (age range 30-90 years; median age 65 years) with a female predominance. It is often diagnosed incidentally as most patients are asymptomatic or present only with vague, dull abdominal pain caused by massive splenomegaly.

Spleen and bone marrow involvement is seen in the majority of cases. The clinical presentation consists of splenomegaly (75% cases); anemia, thrombocytopenia, or leukocytosis (25% cases); autoimmune hemolytic anemia (10-15% cases); hepatomegaly (30% cases); peripheral blood involvement with villous lymphocytes (55-65% cases); abdominal lymphadenopathy (25% cases); peripheral lymphadenopathy (17% cases); and serum paraproteinemia (10-28% cases). A small percentage of cases are associated with Hepatitis C.

This low magnification view of SMZL shows replacement of preexisting lymphoid follicles of splenic white pulp by micronodular tumoral infiltrates.

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