Prognosis and treatment of Waldenström’s macroglobulinemia

Waldenström’s macroglobulinemia is classified by the World Health Organization (WHO) as one of the mature B cell lymphomas. It is a rare clinical entity characterized by a serum monoclonal IgM with morphological evidence of lymphoplasmacytic lymphoma and a specific immunophenotype. Due to the scarcity of the disease, the most optimal therapeutic strategy has not yet been identified. There are, however, a number of active agents used in the treatment of this disease. In this paper we will examine the prognostication of the disease, with emphasis on the International Prognostic Scoring System for Waldenström’s macroglobulinemia (IPSSWM), and outline current therapeutic options.