Waldenström macroglobulinemia: 2013 update on diagnosis, risk stratification, and management.

摘要: Disease Overview Waldenstrom macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, and lymphadenopathy. Diagnosis The presence of IgM protein associated ≥10% clonal cells in bone marrow confirms the diagnosis. Risk Stratification Age, hemoglobin level, platelet count, β2 microglobulin, concentrations are characteristics required for prognosis. Risk-Adapted Therapy Not all patients who fulfill WM criteria require therapy; these can be observed until symptoms develop. Rituximab-based therapy used virtually US combined alkylating agent or purine nucleoside analog (or both). The preferred Mayo Clinic nonstudy therapeutic induction rituximab, cyclophosphamide, dexamethasone. Future stem cell transplantation should considered selection. Management Refractory Disease Bortezomib, thalidomide, everolimus, lenalidomide, bendamustine have been shown to activity WM. Given WM's natural history, reduction complications will priority future treatment trials. Am. J. Hematol. 88:703–711, 2013. © 2013 Wiley Periodicals, Inc.