Plasma Cell Leukemia: a Highly Aggressive Monoclonal Gammopathy with a Very Poor Prognosis

Overview

Journal Int J Hematol

Specialty Hematology

Date 2009 Mar 28

PMID 19326058

Citations 13

Authors

Victor H Jimenez-Zepeda

Virginia J Dominguez-Martinez

Affiliations

Soon will be listed here.

Abstract

Plasma cell leukemia (PCL) is an aggressive variant of multiple myeloma and is characterized by the presence of >20% and/or an absolute number of greater 2 x 10(9)/L plasma cells circulating in the peripheral blood. PCL represents approximately 2-4% of all MM diagnosis and exists in two forms: primary PCL (PPCL, 60% of cases) presents de novo, whereas secondary PCL (SPCL, accounts for the remaining 40%) consists of a leukemic transformation in patients with a previously diagnosed MM. Because the mechanisms contributing to the pathogenesis of PCL are not fully understood, immunophenotyping, genetic evaluation (conventional karyotype, FISH, GEP and array-CGH), and immunohistochemistry are really important tools to investigate why plasma cells escape from bone marrow and become highly aggressive. Since treatment with standard agents and steroids is poorly effective, a combination of new drugs as part of the induction regimens and bone marrow transplant (autologous and allogeneic approaches) could nearly overcome the poor prognosis exhibited by PCL patients.

Citing Articles

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Oligosecretory Primary Plasma Cell Leukemia with Atypical Morphological Abnormality.

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