Primary plasma cell leukaemia: a report of 18 cases

Primary plasma cell leukaemia (P-PCL) is a variant of multiple myeloma (MM) first diagnosed in the leukemic phase, with >2000/mm(3) circulating plasma cells (PCs) and plasmacytosis >20% of the white cell count. We investigate d the clinical characteristics, therapy, immunophenotype and prognosis fact ors of 18 patients. Common features at diagnosis were asthenia (seven patie nts), renal insufficiency (ten patients), bone pain (seven patients), splen omegaly or hepatomegaly (five patients). Hypercalcemia was present at diagn osis in seven patients and was the most potent poor prognosis factor (P < 0 .05). Most patients (16 out of 18) were treated with an anthracyclin contai ning regiment; complete remission was attained in one patient and partial r emission in 11 patients while six patients had no response. The median surv ival time from diagnosis was 7 months (2-12, 95% confidence interval), but response to treatment had favorable predictive value (P < 0.05). The PCs we re usually positive for mature B-cell markers (PCA-1, CD38). They expressed integrins which may increase their binding to endothelial cells and thus p articipate in PCL physiopathology by favoring plasmocyte extramedullary spr ead. (C) 2001 Elsevier Science Ltd. All rights reserved.