Published online by Cambridge University Press: 10 January 2011
Introduction
Waldenstrom's macroglobulinemia (WM) is a distinct clinicopathologic 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 12.1). This condition is considered to correspond to the lymphoplasmacytic lymphoma (LPL) as defined by the Revised European–American Lymphoma (REAL) and World Health Organization (WHO) classification systems. 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. The incidence rate for WM is higher among Caucasians, with African descendants representing only 5% of all patients. Genetic factors appear to be an important factor to the pathogenesis of WM. Approximately 20% of WM patients have an Ashkenazi (Eastern European) Jewish ethnic background, and there have been numerous reports of familiar disease, including multigenerational clustering of WM and other B-cell lymphoproliferative diseases. In a recent study, approximately 20% of 257 serial WM patients presenting to a tertiary referral had a first-degree relative with either WM or another B-cell disorder. Frequent familiar association with other immunologic disorders in healthy relatives, including hypogammaglobulinemia and hypergammaglobulinemia (particularly polyclonal IgM), autoantibody (particularly to thyroid) production, and manifestation of hyperactive B cells have also been reported.
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