Plasma cell leukemia (PCL) is a rare and aggressive variant of myeloma characterized by the presence of circulating plasma cells. It is classi?ed 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 ?ndings. The clinical course is aggressive with short remissions and survival duration. The diagnosis is based upon the percentage (X20%) and absolute number (X2 x 109/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 de?nitions and better understanding of the pathogenesis of PCL.
C Fernandez de Larrea, RA Kyle, BGM Durie, H Ludwig, S Usmani, DH Vesole, R Hajek, JF San Miguel, O Sezer, P Sonneveld, SK Kumar, A Mahindra, R Comenzo, A Palumbo, A Mazumber, KC Anderson, PG Richardson, AZ Badros, J Caers, M Cavo, X LeLeu, MA Dimopoulos, CS Chim, R Schots, A Noeul, D Fantl, U-H Mellqvist, O Landgren, A Chanan-Khan, P Moreau, R Fonseca, G Merlini, JJ Lahuerta, J Blade, RZ Orlowski and JJ Shah on behalf of the International Myeloma Working Group
Leukemia advance online publication, 4 January 2013; doi:10.1038/leu.2012.336