Waldenström's macroglobulinemia is a B-cell malignancy characterized by a lymphoplasmacytic infiltration into the bone marrow and a monoclonal immunoglobulin M (IgM) protein in the serum. It remains incurable despite advances in current therapy, and the decision to treat patients as well as the choice of treatment administered can be rather complex. Management of this disease should utilize a risk-adapted approach. Patients with smoldering or asymptomatic Waldenström's macroglobulinemia with preserved hematological function should be observed without initial therapy. Symptomatic patients with modest hematological compromise, IgM-related neuropathy requiring therapy or hemolytic anemia unresponsive to corticosteroids should receive standard doses of rituximab alone without maintenance therapy. Patients who have significant constitutional symptoms, profound hematological compromise, symptomatic bulky disease or hyperviscosity should be treated with combination therapy. Any patient with symptoms of hyperviscosity should be treated with plasmapheresis. For patients who relapse after an initial response to therapy of greater than two years in duration, the original therapy should be repeated. For patients who had an inadequate response to initial therapy or a response of less than two years duration, an alternative agent or combination should still be used. An autologous stem cell transplant should be considered in all eligible patients with relapsed disease.
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