Richter's transformation of lymphoma complicating Gaucher's disease

A case of lymphoma in a middle-aged woman with Gaucher's disease, a mu-kappa monoclonal gammopathy, peripheral lymphocytosis, and massive splenomegaly is described. There was no evidence of Gaucher's disease in the splenectomy specimen. Instead, the splenic architecture was effaced by a sm...

Full description

Saved in:
Bibliographic Details
Published inHematologic pathology Vol. 3; no. 2; p. 91
Main Authors Paulson, J A, Marti, G E, Fink, J K, Sato, N, Schoen, M, Karcher, D S
Format Journal Article
LanguageEnglish
Published United States 1989
Subjects
B-Lymphocytes
Biomarkers - analysis
Biopsy
Bone Marrow - pathology
Cell Transformation, Neoplastic - pathology
Female
Flow Cytometry
Gaucher Disease - complications
Gaucher Disease - pathology
Humans
Immunohistochemistry
Lymphoma - complications
Lymphoma - pathology
Middle Aged
Phenotype
Spleen - pathology
Online AccessGet more information

Cover

More Information
Summary:A case of lymphoma in a middle-aged woman with Gaucher's disease, a mu-kappa monoclonal gammopathy, peripheral lymphocytosis, and massive splenomegaly is described. There was no evidence of Gaucher's disease in the splenectomy specimen. Instead, the splenic architecture was effaced by a small lymphocytic lymphoma (SLL), with a few foci of large cell lymphoma (LCL). Immunoperoxidase studies showed that both lymphomatous components had a mu-kappa immunophenotype. Flow cytometric analysis of peripheral blood and spleen lymphocyte surface markers confirmed monoclonality. The intensity of surface immunoglobulin expression and the results of other cell marker studies in the SLL component were consistent with a stage of differentiation beyond that typical of chronic lymphocytic leukemia (CLL). The presence of abundant cytoplasmic immunoglobulin in the SLL and the association with monoclonal gammopathy were more consistent with Waldenström's macroglobulinemia or plasmacytoid lymphocytic lymphoma. The immunohistochemical and flow cytometric findings suggest that the SLL component evolved clonally or underwent Richter's transformation to LCL. Although Gaucher's disease has been associated with a variety of B-cell disorders, the subsequent transformation to a high-grade lymphoma has not been previously reported.
ISSN:0886-0238