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Plasma cell leukaemia and other aggressive plasma cell malignancies
Author(s) -
Sher Taimur,
Miller Kena C.,
Deeb George,
Lee Kelvin,
ChananKhan Asher
Publication year - 2010
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2010.08157.x
Subject(s) - multiple myeloma , medicine , plasma cell , plasma cell leukemia , bortezomib , lenalidomide , plasma cell myeloma , proteasome inhibitor , plasma cell neoplasm , chemotherapy , radiation therapy , stem cell , cancer research , oncology , pathology , plasmacytoma , biology , genetics
Summary Extramedullary plasma cell cancers, such as plasma cell leukaemia (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.