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Waldenström Macroglobulinemia

Paraproteinemia and Related Disorders, Page: 211-228
2022
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Book Chapter Description

Waldenström macroglobulinemia (WM) is a rare, low-grade lymphoplasmacytic lymphoma involving the bone marrow, lymph nodes, and spleen with monoclonal immunoglobulin M (IgM) protein secretion (Yun et al., Clin Lymphoma Myeloma Leuk. 17:252–62, 2017). Other low-grade lymphoproliferative disorders may also present with IgM monoclonal protein and should be excluded. Although asymptomatic in some cases, patients may suffer from cytopenia, lymphadenopathy, splenomegaly, or hepatomegaly. Raised serum monoclonal IgM can result in hyperviscosity syndrome. Approximately 1000–1500 new WM cases are diagnosed every year in the United States (Sekhar et al., Leuk Lymphoma. 53:1625–6, 2012). Although incurable, treatment is needed in symptomatic patients. Plasmapheresis is used in case of hyperviscosity. Alkylating agents, nucleoside analogs, anti-CD20 monoclonal antibodies, and/or Bruton tyrosine kinase inhibitors (BTKi), among other agents, single or in combination, have all been used to control clinical manifestations and reduce IgM levels.

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