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POEMS Syndrome and Small Lymphocytic Lymphoma Co-Existing in the Same Patient: A Case Report and Review of the Literature

Journal: Annals of Hematology & Oncology (Vol.2, No. 1)

Publication Date:

Authors : ; ; ;

Page : 1-4

Keywords : POEMS; CLL/SLL; Clonality; Gene rearrangement;

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The coexistence of B-cell Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL) and Plasma Cell Dyscrasias (PCD) has rarely been reported. The patient described herein presented with a clinical course resembling POEMS syndrome. The histopathological evaluation of the bone marrow biopsy established the presence of an osteosclerotic plasmacytoma despite the absence of monoclonal protein in the peripheral blood. Cytochemical analysis of the plasmacytoma demonstrated monotypic expression of lambda (λ) light chains, a typical finding associated with POEMS syndrome. A subsequent lymph node biopsy performed to rule out Castleman's disease led to an incidental finding of B-CLL/SLL predominantly involving the B-zone of the lymph node. The B-CLL population expressed CD19, CD20, CD23, CD5, HLA-DR, and kappa (κ) surface light chains. To the best of our knowledge, a simultaneous manifestation of CLL/SLL and POEMS has not been previously reported in the literature. The expression of a different immunoglobulin (Ig) light chain on the plasmacytoma (λ) and CLL (κ) suggested a biclonal B-cell origin. In our patient, a definite clonal relationship between the two neoplasms by Ig heavy chain gene rearrangements could not be established because of the nonsecretory myeloma and absence of CLL lymphocytosis in the peripheral blood.

Last modified: 2016-08-02 20:13:49