Primary Plasma Cell Leukemia Presented as Progressive Paraplegia: A Case Report

Hasmoni, Mohd Hadzri; Fadilah, S A W; Keng, Cheong Soon

doi:10.1097/smj.0b013e31818018c1

Cited by 3 publications

(1 citation statement)

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“…Primary or de novo PCL (PPCL) occurs in individuals without a preceding diagnosis of plasma cell myeloma [2][3][4]. Secondary PCL (SPCL) arises in patients with a history of plasma cell myeloma who have progressed to a leukemic phase [2][3][4][5][6][7][8]. Kyle et al [9] proposed a criteria of uniformity to diagnose this entity, indicating an absolute count of 2 9 10(9)/L or more than 20% of plasma cells in peripheral blood as characteristic of PCL, although some authors consider it as an arbitrary designation [10] (Table 1).…”

Section: Introductionmentioning

confidence: 99%

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

Jiménez-Zepeda

Dominguez-Martinez

2009

Int J Hematol

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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 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.

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