Waldenström's macroglobulinemia: a review of therapy

Waldenstrom's macroglobulinemia is a lymphoplasmacytic disorder characterized by a monoclonal IgM protein, anemia, hepatosplenomegaly, and hyperviscosity. With the increasing use of screening chemistry evaluations, many patients are diagnosed without symptoms and are candidates for observation with no therapeutic intervention until symptoms develop. Plasma exchange can be useful to manage hyperviscosity but does not address the infiltrative process in the bone marrow, which requires cytoreductive therapy. This review covers current regimens that have been used to manage Waldenstrom's macroglobulinemia, including alkylating agents, purine nucleoside analogs, and rituximab. The value of steroids, radiotherapy, stem cell transplantation, and splenectomy is also reviewed. The lack of phase III studies does not permit an algorithm that would be appropriate for all patients. Treatment needs to be individualized based on patient age, the clinical manifestations of Waldenström's, and the patient's potential for developing toxic side effects of the selected treatment regimen.