Waldenström macroglobulinaemia (WM)

Waldenström macroglobulinaemia (WM) has evolved from a clinical syndrome to a distinct clinicopathological entity. Progress is being made in standardization of the disease definition and treatment response criteria, although controversies persist.

According to the Second International Workshop on WM, the disease is defined as a B-cell neoplasm characterized by lymphoplasmacytic infiltration of the bone marrow and a monoclonal immunoglobulin M (IgM) protein.

Disease symptoms are often divided into those related to tumour infiltration and those related to the rheological effects of the monoclonal IgM. The most common symptom is fatigue attributable to anemia. The prognostic factors predictive of survival include the patient's age, ß2-microglobulin level, monoclonal protein level, hemoglobin concentration, and platelet count.

As with other low-grade lymphomas, asymptomatic patients are observed only, with treatment reserved for symptomatic patients most common fatigue attributable to anemia. There is no standard treatment for WM and choices include steroids, rituximab, alkylating agents, purine nucleoside analogues, alone or in combination, as well as autologous peripheral blood stem cell transplant in eligible patients. Novel treatments, such as bortezomib are being evaluated.

Studies involving combination chemotherapy are ongoing, and preliminary results are encouraging. No specific agent or regimen has been shown to be superior to another for treatment of WM.