Abstract
The treatment of auto-immune thrombocytopenic purpura (AITP) remains unsatisfactory in patients refractory to first-line management such as corticosteroids and/or splenectomy. Those patients usually require unacceptably high doses of corticosteroids to maintain a safe platelet count and thus have refractory AITP. Relativelly new immunosupressive treatment modality is cyclosporin A (CsA) and no large studies involving these drug have been conducted. We treated 7 refractory AITP patients with CsA. Their platelet count were without any therapy below 20x109/L and mostly they had signs of subcutaneous and mucosal bleeding. In 6/7 patients splenectomy was performed earlier. In order to maintain »safe« platelet count, they all needed methylprednisolon (MP), at least 32 mg/daily. So at the time treatment with CsA began, all patients were on MP. During next months MP dosage was tapered or withdrew, according to patients platelet count and treatment efficacy. At the endpoint, in 4/7 patients complete remission (CR) was achieved and later CsA was slowly ceased. 3/7 patients are currently in partial remission (PR) of the disease. Also one of them who has more than 30 years history of the disease is in stable PR, on low dose CsA and low dose MP maintanance therapy. Her platelet count is well above 20x109/L. Among CsA treatment related side effects painful lower limb edema was most frequent.
Based on our experience we suggest, that CsA should be recommended in refractory (chronic) AITP patients, because it shows long-term efficacy and good safety profile and is able to sustain remission at low doses or even after CsA discontinuation.
Multiple myeloma associated with immune thrombocytopenic purpura