Hôpital Pitié-Salpêtrière, 47 bd de l’Hôpital, 75013 Paris, Hôpital Hôtel-Dieu, centre hospitalier universitaire, 63000 Clermont-Ferrand
Waldenström macroglobulinemia (WM) is a rare low-grade lymphoproliferative disorder characterized by a monoclonal lymphoplasmacytic proliferation with a serum monoclonal immunoglobulin (IgM). WM should be regarded as a distinct clinicopathologic entity. Recent works has shown that caryotypic abnormalities with deletion in the long arm of chromosome 6 are observed in 30 % of the patients and that the bone marrow microenvironment interfers with the tumor cells. The clinical manifestations and laboratory abnormalities are related to direct tumor infiltration and to the amount and specific properties of the monoclonal IgM. Treatment decisions should rely on specific clinical and laboratory criteria according to the second international workshop. When therapy is indicated, alkylating agents, nucleoside analogs and rituximab are reasonable choices. Several factors including age, the presence of cytopenia, need of rapid control of the disease, candidacy for autologous stem cell transplantation should be taken into account when choosing the most appropriate primary treatment. 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 second (Athens 2002) and third workshop (Paris 2004) on this rare disease.