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

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%...

Full description

Saved in:
Bibliographic Details
Published inPrzeglad lekarski Vol. 68; no. 6; p. 320
Main Authors Jurczyszyn, Artur, Zawirska, Daria, Skotnicki, Aleksander B
Format Journal Article
LanguagePolish
Published Poland 2011
Subjects
Humans
Leukemia, Plasma Cell - complications
Leukemia, Plasma Cell - diagnosis
Leukemia, Plasma Cell - therapy
Paraproteinemias - etiology
Prognosis
Online AccessGet more information

Cover

More Information
Summary: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.
ISSN:0033-2240