[Treatment of idiopathic thrombocytopenic purpura in adults]

The lack of controlled studies of the treatment of idiopathic thrombocytopenic purpura (ITP), also known as immune thrombocytopenic purpura, means that no evidence-based recommendations are available. ITP is a benign disease and severe hemorrhages are rare. Oral prednisone remains the first-line treatment. In patients with very low platelet counts and significant bleeding, intravenous immunoglobulins and high-dose oral dexamethasone may be an alternative. A validated bleeding score would help physicians use these treatments more effectively. Splenectomy remains the best curative treatment for adults with chronic ITP and platelet counts <30 x 10(9)/L after failure of the first-line treatments. Rituximab is probably the single most effective agent, as well as the least toxic, when splenectomy fails: the short-term response rate is 50% and the sustained-response rate more than 30%. Cytotoxic or other immunosuppressive agents should be reserved for patients with severe disease refractory to both splenectomy and rituximab. Thrombopoietin receptor agonists are a class of new investigational drugs for which promising results have been reported, but more data regarding long-term safety are needed.