Plasma cell leukemia

Abstract

Plasma cell leukemia (PCL) is a rare hematologic malignancy with very poor outcome. It is defined by the presence of >2 × 10(9)/L plasma cells or >20% plasmacytosis of the differential white cell count in the peripheral blood. Primary PCL is first diagnosed in the leukemic phase, while secondary PCL corresponds to the leukemic transformation of a previously diagnosed multiple myeloma (MM). The incidence of PCL ranges between 2-4% of patients with MM and 0.9% of patients with acute leukemia. In this case series, we describe the clinicopathologic, immunophenotypic, and cytogenetic findings of four patients diagnosed with PCL within a ten-year period (2002-2012) at King Faisal Specialist Hospital and Research Centre (General Organization), Riyadh, Saudi Arabia.

Plasma cell leukemia: A rare case

“…Plasma cell leukaemia was first described by Gluzinski and Reichenstein over a century ago. It is a rare form that accounts for only 2-4% of patients with multiple myeloma and 0.9% of all acute leukaemias [1]. It results from monoclonal proliferation of plasma cells and is defined by the presence of plasma cells in the circulating blood with a level of more than 2 giga/L or more than 20% of the leukocytes [1].…”

“…It is a rare form that accounts for only 2-4% of patients with multiple myeloma and 0.9% of all acute leukaemias [1]. It results from monoclonal proliferation of plasma cells and is defined by the presence of plasma cells in the circulating blood with a level of more than 2 giga/L or more than 20% of the leukocytes [1]. If there is any doubt, further investigations should rule out reactive causes such as infection, autoimmune disease or neoplasia.…”

“…Plasma cell leukaemia (PCL) is the rarest but most aggressive variant of monoclonal gammopathies [1]. There are two forms: the primary form occurring de novo in patients without pre-existing multiple myeloma and diagnosed by immediate blood dissemination, and the secondary form, corresponding to a late event in patients with multiple myeloma.…”