Plasma cell leukemia: a rare condition.
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.
Jiménez-Zepeda
Víctor Hugo
VH
Department of Hematology/Oncology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Colonia sección XVI, Tlalpan, CP14000, Mexico. vhugo8762@hotmail.com
Domínguez
Virginia Jeanet
VJ
D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D020732:Cytogenetic Analysis; D003627:Data Interpretation, Statistical; D004921:Erythropoietin / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007952:Leukemia, Plasma Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D008297:Male; D008800:Mexico; D008875:Middle Aged; D009367:Neoplasm Staging; D016609:Neoplasms, Second Primary / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
D004921:Erythropoietin