[Plasma cell leukemia: a highly aggressive monoclonal gammopathy with a very poor prognosis].

Abstract

Plasma cell leukemia (PCL) is an aggressive variant of multiple myeloma and is characterized by the presence of greater then 20% absolute number of 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 very 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 haematopoietic stem cell transplantation (autologous and allogeneic approaches) may overcome the poor prognosis exhibited by PCL patients.

Cite this paper

@article{Jurczyszyn2011PlasmaCL, title={[Plasma cell leukemia: a highly aggressive monoclonal gammopathy with a very poor prognosis].}, author={Artur Jurczyszyn and Daria Zawirska and Aleksander B. Skotnicki}, journal={Przegla̧d lekarski}, year={2011}, volume={68 6}, pages={320-5} }