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Waldenström's macroglobulinemia
Background
Overview
Definition
Waldenstrom's macroglobulinemia is a type of lymphoplasmacytic lymphoma associated with monoclonal IgM gammopathy.
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Pathophysiology
Waldenstrom's macroglobulinemia is caused by an uncontrolled clonal proliferation of terminally differentiated B lymphocytes, which may be due to sporadic or inherited cytogenetic abnormalities.
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Epidemiology
The estimated incidence of Waldenstrom's macroglobulinemia in the US is 0.57 per 100,000 person-years.
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Disease course
Production of IgM paraproteins by Waldenstrom's macroglobulinemia clones leads to clinical manifestations of peripheral neuropathy, systemic amyloidosis, hyperviscosity syndrome, congestive HF, cryoglobulinemia, and death.
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Prognosis and risk of recurrence
The 5-year survival rates for patients with low risk, intermediate risk, and high-risk patients Waldenstrom's macroglobulinemia are 87%, 68%, and 36%, respectively. Treatment-related 1-year mortality rates of up to 44% limit the use of allogeneic transplantation.
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Guidelines
Key sources
The following summarized guidelines for the evaluation and management of Waldenstrom's macroglobulinemia are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2022,2014), the European Society of Medical Oncology (ESMO 2018), and the International Workshop on Waldenstrom's Macroglobulinemia (IWWM 2006).
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