Onkológia 5/2008

WALDENSTRÖM‘S MACROGLOBULINEMIA – EPIDEMIOLOGY, ETHIOPATOGENESIS, DIAGNOSTIC AND TREATMENT

Waldenström‘s macroglobulinemia is characterized by an IgM-expressing lymphoplasmacytoid lymphoma that infiltrates the bone marrow. Therapy is currently reserved for symptomatic patients. There is increasing evidence that purine analogs (PA), and the monoclonal antibody (rituximab, alemtuzumab), active in low-grade lymphoid malignancies are also active in Waldenström‘s macroglobulinemia resistant to alkylating agents. The advances in the biological understanding of Waldenström‘s macroglobulinemia are yielding newer and more targeted therapies for the treatment of this malignancy (proteasome inhibitors, signal inhibitors). High-dose chemotherapy and autologous stem cell transplantation should be considered for all fit patiens with advanced disease where conventional option have failed.

Keywords: Waldenström‘s macroglobulinemia, alkylating agents, nukleoside analogues, rituximab, autologous stem cell transplant