Plasma cell leukemia: advantages of treatment with bortezomib

Plasma cell leukemia is an aggressive variant of multiple myeloma affecting 4% of all cases of the latter. It is characterized by the presence of at least 20% plasma cells circulating in the peripheral blood, equivalent to an absolute number greater than 2,000/mm3. Plasma cell leukemia exits in two forms: primary or de novo plasma cell leukemia (60%) and the secondary form, which consists of a leukemia transformation of a multiple myeloma (40% plasma cell leukemia and 1% multiple myeloma). The disease is rare and has poor prognosis, as there is no effective therapy except the standard melphalan–prednisone combination. We report here on a case of primary plasma cell leukemia diagnosed in the Nice University Hospital’s Onco-hematology Department, which achieved complete response (CR) after eight cycles of bortezomib-cexamethasone followed by maintenance treatment with bortezomib.