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Risk Stratification in Waldenström Macroglobulinemia

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Waldenström’s Macroglobulinemia

Abstract

Waldenström macroglobulinemia (WM) is defined by the production of serum monoclonal immunoglobulin M and lymphoplasmacytic bone marrow infiltration. In addition, a specific molecular abnormality, the MYD88L265P mutation, is present in up to 90 % of patients. WM comprises a heterogeneous population of patients with markedly different evolutions, from indolent courses to rapidly progressing disease. At least 25 % of patients are asymptomatic at diagnosis, and treatment is mandatory in cases of symptomatic disease only. Although, the International Prognostic System for WM has been based on patient populations treated before the availability of many recent agents now used for treatment of WM patients, this index remains the most effective and validated tool for stratifying patients for initial therapy. More research on the biology of the disease is warranted in order to identify additional prognostic factors for predicting outcome, taking the dramatic improvement in survival of patients with symptomatic WM into account.

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Morel, P., Hivert, B. (2017). Risk Stratification in Waldenström Macroglobulinemia. In: Leblond, V., Treon, S., Dimoploulos, M. (eds) Waldenström’s Macroglobulinemia. Springer, Cham. https://doi.org/10.1007/978-3-319-22584-5_19

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