Primary plasma cell leukaemia

Abstract

Among 750 previously untreated patients with multiple myeloma, 27 (4%) presented with plasma cell leukaemia. All but one patient had high tumour mass and, when compared with comparable patients without leukaemia, more frequent extraosseous involvement, thrombocytopenia, high serum lactate dehydrogenase and hypodiploid plasma cells. Most patients also had complex cytogenetic abnormalities. Treatment with standard melphalan-prednisone was ineffective, with a median survival of 2 months, but more intensive chemotherapy induced responses in approximately one-half of the patients, with a median survival of 20 months. Primary plasma cell leukaemia usually results from the proliferation and extramedullary expansion of immature plasma cells and requires prompt and intensive chemotherapy.

Plasma Cell Leukemia: Case Series From a Tertiary Center with Review of Literature

“…Because of the low frequency of PCL, most of the data has come from case reports or small series of cases [2,5,9,10]. In almost all the series median age ranged between 53 and 57 years.…”

“…Because of the low frequency of PCL, most of the literature is either as case reports or a few reviews [2,5,9,10]. Here we are reporting cases of PCL diagnosed from 2004 to 2007 at our center with their clinical and laboratory features.…”

Clinico-Pathological Spectrum of Primary Plasma Cell Leukemia Diagnosed at a Tertiary Care Centre in South India Over 5 Year Period

“…Primary PCL (pPCL) presents de novo in the leukemic phase without a prior history of a plasma cell dyscrasia, while secondary PCL (sPCL) arises in the context of a pre-existing multiple myeloma (MM) [1]. Primary PCL is a rare disorder, representing less than 5% of malignant plasma cell diseases [2] and characterized by plasma cells (PC) circulating in the peripheral blood. Diagnostic criteria were defined by Kyle et al [3] as an absolute plasma cell count of more than 2.0 9 10 9 /l or a relative proportion of greater than 20% of the peripheral blood leukocyte count.…”

Differences in genetic changes between multiple myeloma and plasma cell leukemia demonstrated by comparative genomic hybridization

“…Recently, our group has shown a very high incidence of chromosome 13 monosomies in PCL, 10 but information on the overall chromosomal changes in PCL is still scant. 11 Moreover, to the best of our knowledge CGH has not been used to compare the changes present in PCL and MM patients. In a previous study CGH has been applied to analyze both MM and PCL, but differences have not been explored.…”