Plasma cell leukemia: a rare condition

Ann Hematol. 2006 Apr;85(4):263-7. doi: 10.1007/s00277-005-0054-4. Epub 2006 Jan 14.

Abstract

Plasma cell leukemia (PCL) is a rare lymphoproliferative disorder characterized by a malignant proliferation of plasma cells in the bone marrow and peripheral blood. PCL is also characterized by a fulminant course and poor prognosis. Diagnosis of PCL is established based on Kyle's criteria which include an absolute plasma cell number comprising greater than 20% of peripheral blood cells. PCL has two variants: the primary form presents de novo in patients with no previous history of multiple myeloma (MM) and the secondary form consists of a leukemic transformation in a previously recognized MM. In this paper, we report ten cases of PCL occurring since 1994 to 2005 in a Mexican health institution. Median age at presentation in our study was 58 years, most of them were female (70%). Primary PCL (PPCL) represented 80% and secondary PCL (SPCL) 20%. We describe clinical characteristics, stage, and response to treatment. Interestingly, we report a patient who presented a secondary PCL and acquired activated protein C resistance (APC-R). Additionally, we found an incidence of 20% of venous thrombosis events in two patients with PPCL. Mean survival was 5.9 months (range 2-17) for both PPCL and SPCL. Mean survival for PPCL was 6.75 months and for SPCL 2.0 months, similar to previous literature reports.

MeSH terms

  • Aged
  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage
  • Antineoplastic Combined Chemotherapy Protocols / adverse effects
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Cytogenetic Analysis
  • Data Interpretation, Statistical
  • Erythropoietin / administration & dosage
  • Erythropoietin / adverse effects
  • Erythropoietin / therapeutic use
  • Female
  • Humans
  • Leukemia, Plasma Cell / diagnosis*
  • Leukemia, Plasma Cell / drug therapy
  • Leukemia, Plasma Cell / pathology
  • Male
  • Mexico
  • Middle Aged
  • Neoplasm Staging
  • Neoplasms, Second Primary / diagnosis*
  • Neoplasms, Second Primary / drug therapy
  • Neoplasms, Second Primary / pathology
  • Retrospective Studies
  • Survival Rate
  • Treatment Outcome

Substances

  • Erythropoietin