[Treatment of autoimmune thrombopenic purpura in adults]

Autoimmune thrombocytopenic purpura (AITP) is a common hematological problem. Steroids are the usual first-line treatment but give long-term remission in less than 20% of adults. High doses of human intravenous immunoglobulin G (IVIg) can increase the platelet count in 70 to 80% of patients, but the treatment is expensive and the platelet response usually only transient. Seventy to 80% of the adults have the chronic form of AITP (ie, disease duration of more than 6 months) that only improves on specific treatment. Splenoctomy is the treatment of choice when thrombocytopenia is severe and/or associated with life threatening bleeding, as it cures 60-80% of patients. The best treatment for patients with severe chronic AITP in whom splenectomy is ineffective or contra-indicated is a difficult challenge. In this situation, spontaneous remission is rare and 5% of patients will die from hemmorrhage. However, the treatments so far proposed in refractory chronic AITP (danazol, vinca alcaloids, azathioprine, cyclophosphamide, dapsone, etc) are inconsistently and transiently effective. The aim of the treatments in AITP are thus different in the acute and chronic forms of the disease. In the acute phase, the treatment should quickly increase the platelet count, even if the effect in transient, and aggressive treatments (ie, splenectomy, immunosuppressive drugs) must be avoided since spontaneous remission is possible. On the contrary, splenectomy is the treatment of choice for chronic AITP since it obtains complete persistent recovery in nearly 80% of patients. In the case of unsuccessfullness, treatments should then be administered with the aim to maintain a "safe" platelet count (> 20 to 30 x 10(9)/L).