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 >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.
Jimenez-Zepeda
Victor H
VH
Mayo Clinic Scottsdale, 13400 E Shea Blvd., MCCRB, Suite 300, Scottsdale, AZ, 85259, USA. jimenezzepeda.victor@mayo.edu.
Dominguez-Martinez
Virginia J
VJ
INCMNSZ, Mexico, DF, Mexico.