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

摘要: Plasma cell leukemia (PCL) is a rare and aggressive variant of myeloma characterized by the presence circulating plasma cells. It classified as either primary PCL occurring at diagnosis or secondary in patients with relapsed/refractory myeloma. Primary distinct clinic-pathological entity different cytogenetic molecular findings. The clinical course short remissions survival duration. based upon percentage (X20%) absolute number (X2 � 10 9 /l) cells peripheral blood. proposed that thresholds for be re-examined consensus recommendations are made diagnosis, well as, response progression criteria. Induction therapy needs to begin promptly have high activity leading rapid disease control an effort minimize risk early death. Intensive chemotherapy regimens bortezomib-based recommended followed high-dose autologous stem transplantation if feasible. Allogeneic can considered younger patients. Prospective multicenter studies required provide revised definitions better understanding pathogenesis PCL.