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Plasma cell leukemia: a rare condition

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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.

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Authors and Affiliations

  1. Department of Hematology/Oncology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Colonia sección XVI, Tlalpan, CP14000, Mexico

    Víctor Hugo Jiménez-Zepeda & Virginia Jeanet Domínguez

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  1. Víctor Hugo Jiménez-Zepeda
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  2. Virginia Jeanet Domínguez
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Correspondence to Víctor Hugo Jiménez-Zepeda.

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Jiménez-Zepeda, V.H., Domínguez, V.J. Plasma cell leukemia: a rare condition. Ann Hematol 85, 263–267 (2006). https://doi.org/10.1007/s00277-005-0054-4

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