Abstract
Splenic marginal zone lymphoma (also known as splenic marginal zone B-cell lymphoma) is one of a family of indolent B-cell lymphomas that remain incompletely characterized. These lymphomas can be globally classified into mucosa-associated lymphatic tissue (MALT) lymphoma, also known as extranodal marginal zone lymphoma, and the nonmucosa-associated lymphatic tissue (non-MALT) marginal zone lymphomas, of which there are two: nodal marginal zone lymphoma (NMZL) and splenic marginal zone lymphoma (SMZL) (1,2). While MALT lymphoma has been well described at the molecular and clinical levels, NMZLs and SMZLs are poorly characterized at the molecular level, but remain clinically distinct. NMZL presents with disseminated nodal involvement, but SMZL is often isolated to the spleen and presents with massive splenomegaly (2,3). The author has previously reported a 39% incidence of primary splenic lymphoma in patients with idiopathic splenomegaly (3). SMZL is rare, and a high index of suspicion is necessary to make the diagnosis in a timely manner.
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