Abstract
Waldenström’s Macroglobulinemia (WM) is characterized by the presence of a monoclonal IgM protein in the serum and >10 % lymphoplasmacytic plasma cells in the bone marrow. WM must be differentiated from its precursor conditions: IgM monoclonal gammopathy of undetermined significance (IgM MGUS) and smoldering Waldenström’s macroglobulinemia (SWM) both of which have indolent clinical courses. The former condition is defined as the presence of a monoclonal M protein in the serum <3.0 g/dL and <10 % lymphoplasmacytic cells in the bone marrow without any clinical symptoms. Patients may progress to SWM which is defined as the presence of a serum IgM monoclonal protein ≥3 g/dL and/or ≥10 % bone marrow lymphoplasmacytic infiltration but without constitutional symptoms, symptomatic lymphadenopathy, or hepatosplenomegaly attributable to uncontrolled lymphoplasmacytic proliferation. There are specific indications for instituting therapy for patients with WM. These include the presence of constitutional symptoms such as fever, night sweats, fatigue and weight loss, symptomatic lymphadenopathy or hepatosplenomegaly, or symptomatic anemia, hyperviscosity, or significant thrombocytopenia. Patients with IgM MGUS or SWM should not be treated and should be followed in a “watch and wait” fashion as therapies are toxic, and delaying therapeutic intervention until the development of symptoms in SWM does not adversely impact outcome.
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Kyle, R.A., Ansell, S.M., Kapoor, P. (2017). Indications for Treatment of Waldenström’s Macroglobulinemia. In: Leblond, V., Treon, S., Dimoploulos, M. (eds) Waldenström’s Macroglobulinemia. Springer, Cham. https://doi.org/10.1007/978-3-319-22584-5_20
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DOI: https://doi.org/10.1007/978-3-319-22584-5_20
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