Plasma cell leukaemia and other aggressive plasma cell malignancies

Abstract

Summary Extramedullary plasma cell cancers, such as plasma cell leukemia (PCL) and multiple extramedullary plasmacytomas (MEP) are very aggressive malignancies. These can be primary (de-novo) or secondary due to progressive prior multiple myeloma (MM). Recent reports suggest an increase in incidence of these disorders. Compared to MM, organ invasion is common in PCL, while soft tissue tumors involving the head, neck or paraspinal area are common sites for MEP. Markers of poor prognosis are frequently observed in these extramedullary forms of plasma cell cancers, and survival is significantly inferior compared to patients with MM. Conventional chemotherapeutic and radiotherapy approaches have been employed with variable results. Even high dose chemotherapy with autologous stem cell rescue has not been able to demonstrate consistent improvement in survival outcome. Although not specifically evaluated, novel anti-plasma cell agents, such as the proteasome inhibitor bortezomib, and immunomodulatory drugs, such as lenalidomide, appear to be active against these aggressive cancers. Clinical and translational research directed at improved understanding of disease biology and development of novel therapeutics is urgently needed.

Clinico-Pathological Spectrum of Primary Plasma Cell Leukemia Diagnosed at a Tertiary Care Centre in South India Over 5 Year Period

“…The liver, spleen and other extramedullary sites are more commonly involved than sPCL [11]. Compared to pPCL, patients with sPCL have advanced bone disease [1]. All cases in this series of patients were de novo and had a younger age of presentation, a shorter duration of illness, lesser incidence of bony lytic lesions and greater incidence of extramedullary involvement compared to a series of five cases of PCL from North Indian group of patients reported earlier [6] probably indicating some form of progression of a plasma cell dyscrasia to sPCL in that subgroup.…”

“…Akin to the malignant PC from MM or MGUS patients, malignant PCL cells also express CD38 and CD138 [1]. However, expression of CD20 is more prevalent and there is reduced expression of CD56, CD117, HLA-DR and CD9 antigens on PCL cells compared to MM [5].…”

“…These can be primary (de novo) or secondary. Primary PCL (pPCL) presents de novo in the leukemic phase without a prior history of a plasma cell dyscrasia, while secondary PCL (sPCL) arises in the context of a pre-existing multiple myeloma (MM) [1]. Primary PCL is a rare disorder, representing less than 5% of malignant plasma cell diseases [2] and characterized by plasma cells (PC) circulating in the peripheral blood.…”

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

“…In another study, lenalidomide was given as consolidation/maintenance therapy after PR was obtained in patients previously treated with bortezomib, thalidomide, cyclophosphamide, liposomal doxorubicin, resulting in continued disease control for 20 months in pPCL (Sher et al, 2010).…”

Primary Plasma Cell Leukaemia with Unusual Presentations: A Case Series

“…Pleural effusion is a rare presenting feature of multiple myeloma [6][7][8]. We present three cases of PCL with unusual presentations-first case of primary PCL with aberrant myeloid markers, second case of PCL with aggressive clinical presentation and third case of multiple myeloma which progressed to PCL with myelomatous pleural effusion.…”