Plasma cell leukaemia and other aggressive plasma cell malignancies

Taimur Sher, Kena C. Miller, George Deeb, Kelvin Lee, Asher A Chanan Khan

Research output: Contribution to journalArticle

37 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)418-428
Number of pages11
JournalBritish Journal of Haematology
Volume150
Issue number4
DOIs
StatePublished - Aug 2010
Externally publishedYes

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Keywords

  • bortezomib
  • extramedullary plasmacytoma
  • lenalidomide and thalidomide
  • multiple myeloma
  • plasma cell leukaemia

ASJC Scopus subject areas

  • Hematology

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