Efficacy of bortezomib, lenalidomide and dexamethasone(VRD) in secondary plasma cell leukaemia

Plasma cell leukaemia (PCL) is characterized by circulating plasma cells >2 9 109/l in peripheral blood and/or a peripheral blood plasmacytosis >20%. Primary PCL (pPCL) is defined as a de novo appearance of disease, while secondary PCL (sPCL) corresponds to the leukaemic transformation of a previously diagnosed multiple myeloma (MM) (Albarracin & Fonseca, 2011). sPCL still remains an exceedingly resistant disease with median survivals of 2 months (Tiedemann et al, 2008; Musto et al, 2011a; Pagano et al, 2011). Lenalidomide has been reported to have some activity’ in sPCL although it is short-lived (Benson & Smith, 2007; Musto et al, 2008), while better results have been described when this drug is used as first line therapy in pPCL (Musto et al, 2011b). Bortezomib has been investigated with promising results as single agent or in combination both in pPCL and sPCL.