La maladie de Waldenström ou macroglobulinémie

Thirty per cent of the patients are asymptomatic at diagnosis. The clinical manifestations and laboratory abnormalities are related to direct tumor infiltration and to the amount and the auto-antibody and physical properties of the monoclonal IgM. Median overall survival time ranges from 5 to 8 years, 20% of the patients living more than 10 years and 10% of the patients dying from a cause unrelated to the disease. When therapy is indicated (symptomatic patients), alkylating agents, nucleoside analogs, bortezomib and rituximab are reasonable choices. To conclude, we summarise in this review the most important points on the current understanding of the pathogenesis, clinical features, prognostic factors and therapeutic options developed during the sixth workshop (Venice 2010) and the seventh workshop (Newport 2012) on this rare disease.