Waldenstrom’s macroglobulinemia/lymphoplasmacytic lymphoma

Steven P. Treon, Giampaolo Merlini

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Introduction Waldenstrom’s macroglobulinemia (WM) is a distinct clinicopathological entity resulting from the accumulation, predominantly in the bone marrow, of clonally related lymphocytes, lymphoplasmacytic cells and plasma cells which secrete a monoclonal IgM protein (Figure 15.1)[1]. This condition is considered to correspond to the lymphoplasmacytic lymphoma (LPL) as defined by the World Health Organization classification system[2]. Most cases of LPL are WM, with less than 5% of cases made up of IgA, IgG and non-secreting LPL. Epidemiology and etiology WM is an uncommon disease, with a reported age-adjusted incidence rate of 3.4 per million among males and 1.7 per million among females in the USA, and a geometrical increase with age[3]. The incidence rate for WM is higher among Caucasians, with African descendants representing only 5% of all patients. Genetic factors appear to be important to the pathogenesis of WM. A common predisposition for WM with other malignancies has been raised[4,5], and there have been numerous reports of familiar predisposition, including clustering of family members with WM and other B cell lymphoproliferative diseases[6–10]. In a recent study, 28% of 924 serial WM patients presenting to a tertiary referral had a first or second degree relative with either WM or another B - cell disorder[5]. Frequent familiar association with other immunological disorders in healthy relatives, including hypogammaglobulinemia and hypergammaglobulinemia (particularly polyclonal IgM), autoantibody (particularly to thyroid) production, and manifestation of hyper-responsive B cells have also been reported[10,11].

Original languageEnglish
Title of host publicationMyeloma: Pathology, Diagnosis, and Treatment
PublisherCambridge University Press
Pages190-215
Number of pages26
ISBN (Print)9780511862465, 9781107010574
DOIs
Publication statusPublished - Jan 1 2011

ASJC Scopus subject areas

  • Medicine(all)

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