[Waldenström's macroglobulinemia]

Waldenström's macroglobulinemia is a lymphoproliferative disorder with IgM monoclonal gammopathy. Small lymphocytes, plasmacytoid lymphocytes and plasma cells proliferate in lymph node, bone marrow and spleen. Ig (H) gene rearrangement is observed in one half of the patients. Hyperviscosity syndrome caused by increase of IgM occasionally occurs. Cryoglobulin, consisting of monoclonal IgM is detected in 15% of the patients. Cold agglutinin disease develops in 10% of patients. Plasmapheresis is effective against bleeding, sausage-shaped retinal veins and other manifestations of hyperviscosity syndrome. Cyclophosphamide, melphalan and prednisolone are used for untreated patients. Advanced or refractory cases are treated with the combination of vincristine, doxorubicin, ranimustine and prednisolone. An age greater than 60 years and hemoglobin less than 10 g/dl coincide with lower response rate to chemotherapy and shorter survival.