Splenic marginal zone lymphoma complicated by cold agglutinin disease.

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Splenic marginal zone lymphoma complicated by cold agglutinin disease.

Rinsho Ketsueki. 2017;58(2):132-137

Authors: Ochi K, Yokoyama K, Ohno N, Ota Y, Tojo A

Abstract
Splenic marginal zone lymphoma (SMZL) is a rare low-grade B-cell lymphoma accounting for less than 1% of lymphoid neoplasms and is often associated with autoimmune disorders. A 48-year-old woman presented with severe anemia due to steroid-refractory cold agglutinin disease (CAD), and was referred to our hospital for management of progressive systemic illness and high fever. On admission, she showed elevated serum soluble IL-2R and mild splenomegaly. PET/CT revealed FDG accumulation in the spleen and bone. She was pathologically diagnosed as having splenic marginal zone lymphoma by splenectomy and received 8 cycles of rituximab every 2 weeks, resulting in marked improvement of anemia. Lymphoma cells were positive for CD20/CD11c/CD13, phenotypically compatible with SMZL. We report herein the characteristic features of SMZL, and appropriate procedures for diagnosis and treatment.

PMID: 28321090 [PubMed - in process]

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