Splenomegaly

The surgical removal of the spleen is one of the oldest acknowledged therapies for MMM46 (see Table 49.3), yet has only been palliative in benefit with significant risks (bleeding and infection) of perioperative and long-term complications (i.e., thrombocytosis). If splenec-tomy is only palliative and risky in MMM, should any MMM patient be splenectomized? In a recent retrospective review from our institutional experience with 223 splenectomized MMM patients, we found that patients with certain surgical indications were more likely to experience clinical benefit.9 Specifically, patients whose primary indication for splenectomy was painful splenomegaly experienced durable symptomatic relief and occasional improvement in refractory anemia. However, we found only marginal benefit in patients splenectomized to ameliorate portal hypertension, and adverse outcomes in patients splenectomized for thrombocytopenia. The latter group also experienced increased morbidity and mortality with the procedure. Additionally, postoperative risks of significant thrombo-cytosis were associated with increased preoperative platelet counts. Therefore, our current recommendations are to offer palliative splenectomy to individuals with refractory, severely symptomatic, splenomegaly (unresponsive to hydroxyurea) with aggressive postoperative control of thrombocytosis (with platelet-lowering agents +/— platelet apheresis).

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