Waldenström macroglobulinemia: my way.

Waldenström macroglobulinemia is a lymphoplasmacytic lymphoma. A serum monoclonal IgM protein is required to establish this diagnosis. The clinical features patients develop include normochromic normocytic anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy and signs of hyperviscosity. The International Staging System for Waldenström macroglobulinemia divides patients prognostically based on age, hemoglobin, platelet count, IgM level, and β(2) microglobulin. Some patients with Waldenström macroglobulinemia have a smoldering form and can be observed without intervention. Active agents in the treatment of Waldenström macroglobulinemia include rituximab, chlorambucil, cyclophosphamide, fludarabine, bortezomib, lenalidomide, bendamustine, everolimus, and alemtuzumab. The current preferred Mayo Clinic non-study treatment is rituximab, cyclophosphamide, and dexamethasone. The median survival associated with this disease is now over 10 years.