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An introduction to Waldenström’s macroglobulinemia
Image: IgM antibody – Waldenström's macroglobulinemia
Waldenström's macroglobulinemia (WM; also known as lymphoplasmacytic lymphoma) is a rare, low-grade B cell lymphoproliferative malignancy characterized by small lymphocytes, IgM monoclonal gammopathy and lymphoplasmacytic marrow infiltration.1,2,3
It is named after the Swedish physician Dr. Jan Gösta Waldenström, who first identified the disease.1,3
Waldenström's macroglobulinemia represents around 2% of all hematological malignancies.4 The reported age-adjusted incidence rate is 3.4 per million among the male population and 1.7 per million among the female population in the US and 7.3 and 4.2 per million respectively in the European standard population.5
The malignant cells in Waldenström's macroglobulinemia are thought to originate from cells at a late stage of B-cell differentiation, deriving from B-cell arrest following somatic hypermutation in the germinal center and before terminal plasma cell differentiation.1
It is classified as a Non-Hodgkin Lymphoma (NHL), and has an indolent disease course with slow progression.2 It is generally a disease of the elderly, and the cause of death is typically due more to comorbidities associated with advanced age than to the malignancy.1
Despite an indolent disease course, prompt treatment of Waldenström's macroglobulinemia in symptomatic patients can be essential to address anemia, peripheral neuropathy, and other symptoms, and to avoid irreparable organ damage or fatal complications (such as hyperviscosity syndrome).2,6