Waldenström macroglobulinaemia

Rafael Fonseca, Suzanne Hayman

Research output: Contribution to journalReview article

78 Scopus citations

Abstract

Over time, Waldenström macroglobulinaemia (WM) has evolved conceptually from a clinical syndrome to a distinct clinicopathological entity. Progress is being made in standardization of the disease definition and treatment response criteria, although nosologic controversies persist. According to the Second International Workshop on WM, the disease is defined as a B-cell neoplasm characterized by a lymphoplasmacytic infiltrate in the bone marrow, with an associated immunoglobulin (Ig) M paraprotein. Disease symptoms are often divided into those related to tumour infiltration and those related to the rheological effects of the monoclonal IgM. As with other low-grade lymphomas, asymptomatic patients are observed only, with treatment reserved for symptomatic patients. There is no standard treatment for WM and choices include rituximab, alkylating agents, purine nucleoside analogues, alone or in combination, as well as autologous peripheral blood stem cell transplant in eligible patients. Novel treatments, such as bortezomib, oblimersen sodium, perifosine and others are being evaluated.

Original languageEnglish (US)
Pages (from-to)700-720
Number of pages21
JournalBritish journal of haematology
Volume138
Issue number6
DOIs
StatePublished - Sep 1 2007

Keywords

  • Lymphoplasmacytic lymphoma
  • Waldenström macroglobulinaemia

ASJC Scopus subject areas

  • Hematology

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