Primary plasma cell leukemia: morphologic, immunophenotypic, and cytogenetic features of 4 cases treated with chemotherapy and stem cell transplantation.
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.
Johnson
Malisha R
MR
Department of Hematopathology, University of Texas MD Anderson Cancer Center, Houston, 77030, USA.
Del Carpio-Jayo
Daniel
D
Lin
Pei
P
Giralt
Sergio
S
Anderlini
Paolo
P
Champlin
Richard E
RE
Khouri
Issa F
IF
Vadhan-Raj
Saroj
S
Medeiros
L Jeffrey
LJ
Bueso-Ramos
Carlos E
CE
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
D008562:Membrane Glycoproteins; D011509:Proteoglycans; C508241:SDC1 protein, human; D053668:Syndecan-1; D053667:Syndecans; D051997:ADP-ribosyl Cyclase 1