Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group

• Published in: Leukemia Vol. 27; no. 4; pp. 780 - 791
• Main Authors: 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
• Format: Journal Article Web Resource
• Language: English
• Published: London Nature Publishing Group UK 01.04.2013; Nature Publishing Group
• Subjects: 692/420; 692/699/67/1059; 692/699/67/1990/283; Autografts; Bortezomib; Cancer Research; Cancer therapies; Care and treatment; Chemotherapy; Criteria; Critical Care Medicine; Cytogenetics; Diagnosis; Disease control; Disease Progression; Female; Hematology; Hospitals; Human health sciences; Humans; Hématologie; Induction therapy; Intensive; Internal Medicine; Leukemia; Leukemia, Plasma Cell - diagnosis; Leukemia, Plasma Cell - pathology; Leukemia, Plasma Cell - therapy; Lymphoma; Male; Medical diagnosis; Medical prognosis; Medicin och hälsovetenskap; Medicine; Medicine & Public Health; Middle Aged; Multiple myeloma; Myeloma; Oncology; Pathogenesis; Patients; Peripheral blood; Plasma; Plasma cell diseases; Plasma cell leukemia; Plasma cells; review; Sciences de la santé humaine; Stem cell transplantation; Stem cells; Transplantation; Working groups; Italy; Belgium; France; United States > US; Baltimore Maryland; Spain; myeloma; cytogenetics; bortezomib; prognosis; plasma cell leukemia; transplantation
• ISSN: 0887-6924; 1476-5551; 1476-5551
• DOI: 10.1038/leu.2012.336

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.