Review Article

International Journal of Hematology

, Volume 89, Issue 3, pp 259-268

First online:

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

  • Victor H. Jimenez-ZepedaAffiliated withMayo Clinic Scottsdale Email author 
  • , Virginia J. Dominguez-MartinezAffiliated withINCMNSZ

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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 × 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.


Plasma cell leukemia Multiple myeloma Bone marrow transplant and prognosis