Waldenström's macroglobulinemia

Waldenström's macroglobulinemia is an unusual low-grade lymphoplasmacytic lymphoma characterized by the production of monoclonal IgM. The clinical manifestations associated with WM can be classified as those related to direct tumor infiltration, by the amount and specific properties of circulating IgM, and by the deposition of IgM in various tissues. Asymptomatic patients should be followed without treatment. The management of the disease relies on the administration of systemic chemotherapy to reduce tumor load and on the application of plasmapheresis to remove circulating IgM. Standard treatment consists of oral chlorambucil, which induces response in at least 50% of patients, resulting in a median survival of approximately 5 years. Nucleoside analogues (cladribine, fludarabine) are effective in most previously untreated patients. These agents are the treatment of choice for patients with disease resistant to alkylating agents. New treatment approaches include high-dose therapy with stem-cell support and administration of monoclonal anti-CD20 antibodies.