title: Primary plasma cell leukemia: morphologic, immunophenotypic, and cytogenetic features of 4 cases treated with chemotherapy and stem cell transplantation.
abstract: Plasma cell leukemia (PCL) is a neoplastic disorder of plasma cells of which there are 2 forms, primary PCL and secondary PCL, the latter occurring in patients with a history of plasma cell myeloma. We describe 4 patients with primary PCL. In all cases, the bone marrow aspirate smears and biopsy specimens demonstrated a diffuse infiltrate of atypical plasma cells that were difficult to classify using morphologic criteria alone. Immunophenotypic studies showed that each case was positive for plasma cell-associated antigens (cytoplasmic immunoglobin, CD38, or CD138) and negative for CD20. Of 4 cases, 3 had complex karyotypes, including the t(11;14)(q13;q32) or del(11)(q13). Despite chemotherapy and the use of novel therapeutic agents and stem cell transplantation, all 4 patients had short survival.
authors.affiliation: Department of Hematopathology, University of Texas MD Anderson Cancer Center, Houston, 77030, USA.
authors.forename: Malisha R
authors.initials: MR
authors.lastname: Johnson
authors.forename: Daniel
authors.initials: D
authors.lastname: Del Carpio-Jayo
authors.forename: Pei
authors.initials: P
authors.lastname: Lin
authors.forename: Sergio
authors.initials: S
authors.lastname: Giralt
authors.forename: Paolo
authors.initials: P
authors.lastname: Anderlini
authors.forename: Richard E
authors.initials: RE
authors.lastname: Champlin
authors.forename: Issa F
authors.initials: IF
authors.lastname: Khouri
authors.forename: Saroj
authors.initials: S
authors.lastname: Vadhan-Raj
authors.forename: L Jeffrey
authors.initials: LJ
authors.lastname: Medeiros
authors.forename: Carlos E
authors.initials: CE
authors.lastname: Bueso-Ramos
mesh_terms: D051997:ADP-ribosyl Cyclase 1 / Q000378:metabolism; D000328:Adult; D001853:Bone Marrow / Q000473:pathology; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D007621:Karyotyping; D007952:Leukemia, Plasma Cell / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology*; D008297:Male; D008562:Membrane Glycoproteins / Q000378:metabolism; D008875:Middle Aged; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology; D011509:Proteoglycans / Q000378:metabolism; D033581:Stem Cell Transplantation; D016019:Survival Analysis; D053668:Syndecan-1; D053667:Syndecans
chemical_list: D008562:Membrane Glycoproteins; D011509:Proteoglycans; C508241:SDC1 protein, human; D053668:Syndecan-1; D053667:Syndecans; D051997:ADP-ribosyl Cyclase 1