Splenic marginal zone lymphoma natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Associate Editor(s)-in-Chief: Muhammad Affan M.D.[2] Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [3]

Overview

Splenic marginal zone lymphoma (SMZL) is a rare, slow growing B-cell lymphoma that is mostly asymptomatic at the time of diagnosis. It is commonly diagnosed at an old age. Patients typically have splenomegaly, lymphocytosis or cytopenias. Bone marrow is frequently involved but lymphadenopathy and liver involvement is rare.There are automimmune conditions that may develop in this conditions such autoimmune hemolytic anemia, idiopathic thrombocytopenic purpura, angioedema and von-willebrand disease. It may transform into diffuse large B-cell lymphoma. The prognosis is generally good. Several factors including lymphadenopathy, non-hematopoietic site involvement, histologic transformation affects the prognosis. Low Hemoglobin levels, high lactate dehydrogenase levels, low blood serum albumin levels, and genetic mutations such as mutations in NOTCH2, TP53, KLF2 are associated with poor prognosis among patients with splenic marginal zone lymphoma.

Natural history

Complications

Prognosis

References

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