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n Medical Technology SA - Waldenström macroglobulinaemia : a brief review : peer reviewed review

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Abstract

First described in the 1940s, Waldenström Macroglobulinaemia (WM) over time has developed conceptually from a clinical syndrome to a definitive clinicopathological entity. Progress is being made in standardisation of the disease definition, the overall treatment response criteria and the role of the haematology laboratory in supporting the WM patient, although nosologic disputes persist. The World Health Organisation (WHO), for example, has defined WM as a lymphoplasmacytic lymphoma (LPL) associated with IgM monoclonal gammopathy and bone marrow involvement. The latest guidelines from the British Committee for Standards in Haematology, Royal College of Pathologists (BCSH) define WM as an LPL which is a slowly progressive, clonal disorder of mature B cells, with features of plasma cell differentiation. Paraproteinaemia (usually IgM) is common and may give rise to hyperviscosity. The clonal expansions of these post-germinal centre lymphoid cells express a number of immunophenotypic markers that include: CD19, CD20 and surface IgM. These markers along with a number of others can be used to aid diagnosis. Disease symptoms are often divided into those related to tumour infiltration and those related to the effects of the monoclonal IgM. As with the other low-grade lymphomas, asymptomatic patients are observed only, with treatment reserved for those symptomatic patients. WM is a rare disorder that only comprises around 2% of all haematological malignancies. The haematology laboratory's role in diagnosis and monitoring the disease has grown over the years and this is best understood by examining the pathophysiology/biology, diagnosis and treatment of WM.

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/content/medtech/28/1/EJC157716
2014-06-01
2016-12-07
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