Splenic Irradiation

Splenic irradiation should be considered as an alternative to splenectomy in patients who are unfit for surgery. Responses are transient, although some patients may experience prolonged relief, and life-threatening cytopenias may be an unpredictable complication.35 Radiotherapy, however, remains the treatment of choice for peritoneal and pleural extramedullary hematopoeisis, as well as for involvement of vital organs, including the central nervous system.

Experimental Therapies

Bone marrow transplantation is the only therapeutic modality that has been shown to prolong survival. As a result, a number of alternative therapies have been investigated, including vitamin D analogues, alpha interferon, erythropoietin, 2-chlorodeoxyadenosine, etidronate, anagrelide, perfenidone, and suramin. Unfortunately, these agents have not been documented to have a significant clinical benefit.36 Interest, however, has been shown recently in the antiangiogenic agent thalidomide, as CMIF is characterized by a marked neovascularization.37 Side effects are a significant problem and include somnolence, paresthesia, and abdominal discomfort. Thalidomide appears effective in reducing splenomegaly and improving anemia in patients who can tolerate the drug. Platelet counts can increase, and the drug should be used with caution in patients with thrombocythemia.38

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