Περίληψη:
Plasma cell leukemia (PCL) is a rare and aggressive variant of myeloma characterized by the presence of circulating plasma cells. It is classified as either primary PCL occurring at diagnosis or as secondary PCL in patients with relapsed/refractory myeloma. Primary PCL is a distinct clinic-pathological entity with different cytogenetic and molecular findings. The clinical course is aggressive with short remissions and survival duration. The diagnosis is based upon the percentage (≥20%) and absolute number (≥2 × 10 9/l) of plasma cells in the peripheral blood. It is proposed that the thresholds for diagnosis be re-examined and consensus recommendations are made for diagnosis, as well as, response and progression criteria. Induction therapy needs to begin promptly and have high clinical activity leading to rapid disease control in an effort to minimize the risk of early death. Intensive chemotherapy regimens and bortezomib-based regimens are recommended followed by high-dose therapy with autologous stem cell transplantation if feasible. Allogeneic transplantation can be considered in younger patients. Prospective multicenter studies are required to provide revised definitions and better understanding of the pathogenesis of PCL. © 2013 Macmillan Publishers Limited All rights reserved.
Συγγραφείς:
Fernández De Larrea, C.
Kyle, R.A.
Durie, B.G.M.
Ludwig, H.
Usmani, S.
Vesole, D.H.
Hajek, R.
San Miguel, J.F.
Sezer, O.
Sonneveld, P.
Kumar, S.K.
Mahindra, A.
Comenzo, R.
Palumbo, A.
Mazumber, A.
Anderson, K.C.
Richardson, P.G.
Badros, A.Z.
Caers, J.
Cavo, M.
Leleu, X.
Dimopoulos, M.A.
Chim, C.S.
Schots, R.
Noeul, A.
Fantl, D.
Mellqvist, U.-H.
Landgren, O.
Chanan-Khan, A.
Moreau, P.
Fonseca, R.
Merlini, G.
Lahuerta, J.J.
Bladé, J.
Orlowski, R.Z.
Shah, J.J.
Λέξεις-κλειδιά:
beta1 integrin; bortezomib; carmustine; cisplatin; dexamethasone; doxorubicin; gelatinase B; glucocorticoid; interleukin 3; interleukin 6 receptor; melphalan; prednisone; protein p16; vascular cell adhesion molecule 1; vincristine, autologous stem cell transplantation; cancer chemotherapy; cancer control; cancer prognosis; cancer recurrence; cancer regression; cancer survival; cause of death; cell count; consensus; disease course; drug efficacy; fluorescence in situ hybridization; gene expression profiling; gene sequence; hematopoietic stem cell transplantation; human; immunophenotyping; long term survival; maintenance therapy; overall survival; plasma cell; plasma cell leukemia; priority journal; review; treatment response, Disease Progression; Female; Humans; Leukemia, Plasma Cell; Male; Middle Aged
