Splenic irradiation in the management of Waldenstrom macroglobulinemia.

摘要

The fourth international Workshop on Waldenstrom's macroglobulinemia recently published guidelines on the management of this disease. In first line treatment, the following combination regimes are recommended - nucleoside analogues plus alkylators such as cladribine or fludarabine plus cyclophosphamide; nucleoside analogues plus alkylators plus rituximab such as cladribine, cyclophosphamide and rituximab; cyclophosphamide-based combination therapy plus rituximab such as CHOPR; immunomodulatory drugs such as thalidomide plus rituximab. Recommended single agent therapies include rituximab, an alkylating agent or a nucleoside analogue [1,2]. Unfortunately, even with combination therapies complete response rates remain low and many studies do not yet have mature follow-up data [2]. Older studies of single agent treatments do not report standardised end-points, and specifically do not report on pathological complete response in bone marrow. The recommendations do not discuss the use of splenic irradiation and relatively little has been published on the use of splenic irradiation in the treatment of this disease [2,3]. In our experience, with careful monitoring, splenic irradiation can be a well tolerated, effective treatment. We report on a case of Waldenstrom macroglobulinemia treated with splenic irradiation resulting in undetectable disease pathologically and immunologically 18 years following initial treatment.