title: Primary plasma cell leukaemia: a report of 18 cases.
abstract: 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 investigated the clinical characteristics, therapy, immunophenotype and prognosis factors of 18 patients. Common features at diagnosis were asthenia (seven patients), renal insufficiency (ten patients), bone pain (seven patients), splenomegaly or hepatomegaly (five patients). Hypercalcemia was present at diagnosis 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 containing regiment; complete remission was attained in one patient and partial remission in 11 patients while six patients had no response. The median survival time from diagnosis was 7 months (2--12, 95% confidence interval), but response to treatment had favorable predictive value (P<0.05). The PCs were usually positive for mature B-cell markers (PCA-1, CD38). They expressed integrins which may increase their binding to endothelial cells and thus participate in PCL physiopathology by favoring plasmocyte extramedullary spread.
authors.affiliation: Département d'Hématologie, Institut Paoli-Calmettes, 232 bd de Sainte Marguerite, 13009 Marseille, France. costellor@marseille.fnclcc.fr
authors.forename: R
authors.initials: R
authors.lastname: Costello
authors.forename: D
authors.initials: D
authors.lastname: Sainty
authors.forename: R
authors.initials: R
authors.lastname: Bouabdallah
authors.forename: J P
authors.initials: JP
authors.lastname: Fermand
authors.forename: A
authors.initials: A
authors.lastname: Delmer
authors.forename: M
authors.initials: M
authors.lastname: Diviné
authors.forename: J P
authors.initials: JP
authors.lastname: Marolleau
authors.forename: J A
authors.initials: JA
authors.lastname: Gastaut
authors.forename: D
authors.initials: D
authors.lastname: Olive
authors.forename: P
authors.initials: P
authors.lastname: Rousselot
authors.forename: P
authors.initials: P
authors.lastname: Chaïbi
mesh_terms: D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007952:Leukemia, Plasma Cell / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D016019:Survival Analysis; D016896:Treatment Outcome