title: Plasma cell leukemia: concepts and management.
abstract: Plasma cell leukemia (PCL) is a rare and aggressive plasma cell dyscrasia. Patients with PCL have a very poor prognosis with median survival measured in months. PCL can present de novo or following a prodrome of plasma cell myeloma. Patients with PCL tend to present with aggressive clinical features, such as extramedullary disease, bone marrow failure, advanced stage disease and expression of distinct immunophenotypic markers, such as lack of CD56 and presence of CD20. Historically, the treatment of PCL has primarily been palliative, with only a small minority of patients achieving a durable remission. The impact of newer agents, such as bortezomib and lenalidomide, in conjunction with autologous and allogeneic stem cell transplantation is uncertain, but emerging data suggest that use of these modalities may help improve the poor prognosis of patients with PCL.
authors.affiliation: Department of Medicine, Division of Hematology, Stanford Cancer Center, 875 Blake Wilbur Drive, Room 2329, Stanford, CA 94305-5821, USA.
authors.forename: Michaela
authors.initials: M
authors.lastname: Liedtke
authors.forename: Bruno C
authors.initials: BC
authors.lastname: Medeiros
mesh_terms: D000368:Aged; D018951:Antigens, CD20 / Q000032:analysis; D000970:Antineoplastic Agents / Q000008:administration & dosage* / Q000627:therapeutic use; D001897:Boronic Acids / Q000008:administration & dosage* / Q000627:therapeutic use; D000069286:Bortezomib; D019002:CD56 Antigen / Q000032:analysis; D006801:Humans; D000077269:Lenalidomide; D007952:Leukemia, Plasma Cell / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D009101:Multiple Myeloma / Q000401:mortality / Q000628:therapy; D016609:Neoplasms, Second Primary / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D010166:Palliative Care; D010265:Paraproteinemias / Q000503:physiopathology / Q000628:therapy*; D011379:Prognosis; D011719:Pyrazines / Q000008:administration & dosage* / Q000627:therapeutic use; D033581:Stem Cell Transplantation; D013792:Thalidomide / Q000008:administration & dosage / Q000031:analogs & derivatives* / Q000627:therapeutic use; D014182:Transplantation, Autologous; D016896:Treatment Outcome
chemical_list: D018951:Antigens, CD20; D000970:Antineoplastic Agents; D001897:Boronic Acids; D019002:CD56 Antigen; D011719:Pyrazines; D013792:Thalidomide; D000069286:Bortezomib; D000077269:Lenalidomide