title: Plasma cell leukemia: from biology to treatment.
abstract: Plasma cell leukemia (PCL) is a very aggressive and rare form of malignant monoclonal gammopathy characterized by the presence of plasmocytes in peripheral blood. It is classified as primary PCL occuring 'de novo', or as secondary PCL in patients with relapsed/refractory multiple myeloma. Primary PCL is a distinct clinicopathological entity from myeloma with different cytogenetic abnormalities and molecular findings, which are usually found only in advanced multiple myeloma. The clinical course is aggressive with short remissions and reduced overall survival. The diagnostic criteria are based on the percentage (>20%) and absolute number (2 × 10(9) /L) of plasma cells in peripheral blood. After establishing diagnosis, induction therapy should begin promptly which is aimed to rapid disease control and to minimize the risk of early death. Intensive chemotherapy regimens and bortezomib-based regimens, followed by high-dose therapy with autologous stem cell transplantation, are recommended. Allogeneic transplantation can be considered in younger patients. This article reviews recent knowledge of this hematological malignancy that is associated with a very poor prognosis.
authors.affiliation: University Hospital Ostrava, Department of Haematooncology, Ostrava, Czech Republic.
authors.forename: Tomas
authors.initials: T
authors.lastname: Jelinek
authors.affiliation: University of Ostrava, Faculty of Medicine, Ostrava, Czech Republic.
authors.forename: Fedor
authors.initials: F
authors.lastname: Kryukov
authors.affiliation: University Hospital Brno, Department of Clinical Haematology, Brno, Czech Republic.
authors.forename: Lucie
authors.initials: L
authors.lastname: Rihova
authors.affiliation: University Hospital Ostrava, Department of Haematooncology, Ostrava, Czech Republic.|University of Ostrava, Faculty of Medicine, Ostrava, Czech Republic.|University Hospital Brno, Department of Clinical Haematology, Brno, Czech Republic.
authors.forename: Roman
authors.initials: R
authors.lastname: Hajek
mesh_terms: D015703:Antigens, CD / Q000235:genetics / Q000378:metabolism; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D000069286:Bortezomib / Q000627:therapeutic use*; D002869:Chromosome Aberrations; D018450:Disease Progression; D015870:Gene Expression; D006801:Humans; D060828:Induction Chemotherapy / Q000379:methods; D007952:Leukemia, Plasma Cell / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D009101:Multiple Myeloma / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D010950:Plasma Cells / Q000473:pathology; D011379:Prognosis; D033581:Stem Cell Transplantation; D016019:Survival Analysis; D014182:Transplantation, Autologous; D014184:Transplantation, Homologous
chemical_list: D015703:Antigens, CD; D000069286:Bortezomib
keywords: bone marrow transplantation; bortezomib; multiple myeloma; plasma cell leukemia