Response and Progression Criteria

Survival among patients with pPCL rarely exceeds 12 months and median survival in one large study was 4 months, the Working Group reports. Survival rates at 5 years are never higher than 10%, they report. Poor survival is due in part to the lack of sustainable responses to treatment, they write.

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“There are no specific response criteria for PCL,” they note. The evaluation of response in pPCL “should combine acute leukemia and MM requirements.” Complete response (CR) should be based on the “disappearance of peripheral blood plasma cells and a bone marrow plasma cell count less than 5%” following hematological recovery, they recommend. “Complete clearance by conventional morphology in bone marrow for complete remission is required, and flow cytometry should be necessary to define ‘stringent’ CR. In addition, the high frequency of extramedullary involvement justifies evaluation of the patients by imaging techniques such as magnetic resonance imaging and, particularly, fluorodeoxyglucose positron emission tomography/computed tomography.”

Treatment Recommendations

“The goal of induction therapy is to achieve rapid cytoreduction to minimize the risks contributing to early death,” the Working Group reports. “Intensive chemotherapy with alkylating agents or anthracyclines such as HyperCVAD or PACE regimens and bortezomib-based combinations (VTD-PACE [cisplatin, doxorubicin, cyclophosphamide, and etoposide], HyperCVAD-VD or PAD) can meet these goals.”

Clinical data is limited, but bortezomib “likely” improves patient outcomes and “this drug will likely become the backbone in the treatment of PCL” for fit patients, the Working Group concludes.

High-dose therapy and autologous stem cell transplantation appear to improve outcomes for pPCL “but without significant improvement in sPCL,” the Working Group notes. “The role of consolidation and maintenance therapy needs to be evaluated. The impact of tandem autologous cell transplant and allogeneic transplantation also remains to be defined.”

Among patients younger than age 50, for whom a suitable donor is available, myeloablative allogeneic transplantation should be considered, the authors recommend. “Otherwise, a tandem transplant with an ASCT followed by a reduced-intensity conditioning allogeneic transplantation if a related or an unrelated donor is available can be considered.”

Among patients who are not eligible for HDT/SCT, bortezomib-based induction (MPV, VRD, or VTD) is “the best choice” based on limited data and expert opinion, the consensus guidelines state.


1. De Larrea CF, Kyle RA, Durie BGM, et al. Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group [published online ahead of print January 4, 2013]. Leukemia. Accessed January 15, 2013.

2. Van de Donk NWCJ, Lokhorst HM, Anderson KC, Richardson PG. How I treat plasma cell leukemia. Blood. 2012. Accessed Jan. 15, 2013.