Abstract

Plasma cell leukemia (PCL) is a rare and aggressive plasma cell disorder, characterized by the presence of a peripheral blood absolute plasma cell count of at least 2 × 109/l and more than 20% circulating plasma cells. The prognosis of PCL patients remains poor. Even by using autologous or allogenic transplant procedures, median survival does not exceed 3 years (Saccaro et al., 2005). Thalidomide, bortezomib and lenalidomide (Revlimid) have emerged as high active agents in the treatment of PCL (Johnston and abdalla, 2002; Musto et al., 2007; Finnegan et al., 2006). In particular, Lenalidomide is a structural analogue of thalidomide with similar but more potent biological activity; it is used as first line therapy in MM (Palumbo et al., 2007; Niesvizky et al., 2007), although information regarding its associated use with dexamethasone use as salvage therapy in PCL derives from anecdotal single case reports (Musto et al., 2008). We would like to describe a case of primary PCL with adverse cytogenetic in which excellent response was achieved with the combination of lenalidomide, melphalan, and prednisone as salvage therapy.

Highlights

  • The patient did not respond to the induction therapy and, he underwent two cycles of combination chemotherapy consisting of bortezomib (1.3 mg/m2 d 1, 4, 8, 11), doxorubicin (20 mg d 1.4 ) and dexamethasone (40 mg d -2-1, 2, 3, 4)

  • Plasma cell leukaemia is a rare and aggressive form of multiple myeloma characterized by very poor prognosis

  • A single case report evidenced the effectiveness of the association of prednisone and lenalidomide in relapsed Plasma cell leukemia (PCL) [6]

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Summary

Introduction

Plasma cell leukemia (PCL) is a rare and aggressive plasma cell disorder, characterized by the presence of a peripheral blood absolute plasma cell count of at least 2 × 109/l and more than 20% circulating plasma cells. The management decision was to perform autologous stem cell transplantation (ASCT) following adequate stem cell collection with cyclophosphamide and two cycles of combination chemotherapy with vincristine, doxorubicin, and dexamethasone (VAD). The patient did not respond to the induction therapy and, he underwent two cycles of combination chemotherapy consisting of bortezomib (1.3 mg/m2 d 1, 4, 8, 11), doxorubicin (20 mg d 1.4 ) and dexamethasone (40 mg d -2-1, 2, 3, 4).

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