Advances in the treatment of Waldenström's macroglobulinemia

Waldenström's macroglobulinemia is a distinct disorder characterized by a monoclonal immunoglobulin M paraprotein and morphological evidence of lymphoplasmacytic lymphoma. It is relatively rare, accounting for approximately 2% of all hematological malignancies. The aim of treatment for patients with Waldenström's macroglobulinemia should be to improve the quality and duration of life with minimal side effects in the most cost-effective manner. It is not yet clear if achievement of a complete remission confers clinical benefit and it is possible that prolonging therapy to maximal response may increase toxicity without extra benefit. Plasma exchange is indicated for the acute management of patients with severe problems due to a circulating paraprotein. There are no comparative data but alkylating agent-based treatments, combination therapy or purine analogs are all suitable choices for the initial therapy of patients requiring treatment. In younger patients, in whom high-dose treatment is contemplated, there is a role for the use of rituximab; however, it should be administered with caution in patients with high levels of immunoglobulin M paraprotein or signs of hyperviscosity because of the risk of 'flare' in the paraprotein level and consequent adverse clinical events.