Therapy and Prognosis
Treatment of plasma cell leukemia is by supportive care and systemic chemotherapy. Combination of cyclophosphamide, doxorubicin, vincristine, and prednisone (or dexamethasone) can be used. A second combination is the use of cyclophosphamide, dexamethasone, and thalidomide as for myeloma. Another regimen termed VMCP/VBAP uses alternating vincristine, melphalan (M), cyclophosphamide, prednisone/vincristine, carmustine, doxorubicin, and prednisone polychemotherapy. In general, combination chemotherapy has resulted in median survivals of 18 to 20 months compared to 2 to 6 months when single agent therapy is used. There are anecdotal reports of excellent responses and 2- to 3-year disease-free survivals after autologous stem cell transplantation. Autologous transplantation may increase benefit from conventional therapy and high dose melphalan may prolong progression free and overall survival.
Although the clinical and laboratory features of primary and secondary PCL are similar, the response to therapy and overall survival in primary and secondary PCL go from poor to worse. Patients with secondary PCL are usually refractory to chemotherapy and have a poor survival compared with duration (median <2 months).
Novel agents like thalidomide, lenalidomide and bortezomib have been recently used, alone or in combination with conventional chemotherapeutics like melphalan or cyclophosphamide. Two cases had encouraging results with bortezomib. Recent studies demonstrate that velcade (bortezomib)- based regimens offer a significant profit in patients suffering from PCL. More aggressive therapeutic regimens like DT-PACE or protocols used in ALL offer a significant response rate, but with short remission interval.
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