Primary Plasma Cell Leukemia: Clinical, Immunophenotypic, DNA Ploidy, and Cytogenetic Characteristics

摘要: We report on a series of 26 patients diagnosed with primary (de novo) plasma cell (PC) leukemia (PCL) in whom we analyzed the clinicobiologic characteristics disease together immunophenotype, DNA content, proliferative index, and numeric chromosomal aberrations neoplastic PC, compared them 664 multiple myeloma (MM) at diagnosis. The median age, sex ratio, bone lesion extension were similar, but PCL cases displayed higher prevalence clinical stage III, extramedullary involvement, Bence Jones cases, fewer IgA than for MM patients. In addition, according to several prognostic indicators (beta2-microglobulin serum level, proportion S-phase PCs, proteinuria, calcium lactate dehydrogenase [LDH] renal function), incidence adverse factors was significantly versus MM. Immunophenotypic expression similar CD38, CD138, CD2, CD3, CD16, CD10, CD13, CD15, differed from CD56, CD9 HLA-DR, CD117, CD20 antigens. Twenty-two diploid one hypodiploid, while most (57%) showed hyperdiploidy. With fluorescent situ hydridization (FISH) technique, 12 13 aberrations, -13 (86%), +/-1 (57%), +18 (43%), -X women (25%), they lacked usually found such as +3, +6, +9, +11, +15. had lower overall response therapy (38% v 63%, P =.01332). Among patients, trend worse observed treated melphalan prednisone (MP) polychemotherapy. Overall survival (8 36 months, <.0001), it better polychemotherapy MP (18 3 =.0137). By contrast, did not show significant differences treatment used, or Ten variables seemed predict only beta2-microglobulin level PCs retained an independent value multivariate analysis. summary, our study illustrates that display singular phenotypic, cytogenetic lead different evolution