A Scaradavou1, J Bussel. 1. Department of Pediatrics, Cornell Medical Center-New York Hospital, New York, New York 10021, USA.
Abstract
PURPOSE: We evaluate the efficacy of combination medical therapy in the treatment of ITP and AIHA episodes for patients with Evans syndrome. PATIENTS AND METHODS: Five patients with Evans syndrome were followed for a median of 3.8 years and were treated according to our multiagent (IVIG, steroids, vinca alkaloids, androgens, and cyclosporin) protocol. RESULTS: All patients initially received IVIG and IV steroids for either acute hemolysis or thrombocytopenia. IV vinca alkaloids and oral Danazol were added in three patients for ITP. All responded, but two required frequently repeated vinca alkaloid treatments. Both patients treated with vinca alkaloids for severe hemolysis responded. Two patients received oral cyclosporin for refractory ITP; one had a transient response. Splenectomy was not included in our regimen. Two patients underwent splenectomy prior to their referral; both required further therapy. In addition, review of the clinical courses of our patients indicate that the DAT may be a prognostic factor for the chronicity of ITP. CONCLUSION: In view of the high relapse rates after splenectomy and the encouraging results of our pilot study, we suggest that medical treatment with combination agents (IVIG, steroids, vinca alkaloids, androgens and possibly cyclosporin) may provide a useful therapeutic approach to patients with Evans syndrome.
PURPOSE: We evaluate the efficacy of combination medical therapy in the treatment of ITP and AIHA episodes for patients with Evans syndrome. PATIENTS AND METHODS: Five patients with Evans syndrome were followed for a median of 3.8 years and were treated according to our multiagent (IVIG, steroids, vinca alkaloids, androgens, and cyclosporin) protocol. RESULTS: All patients initially received IVIG and IV steroids for either acute hemolysis or thrombocytopenia. IV vinca alkaloids and oral Danazol were added in three patients for ITP. All responded, but two required frequently repeated vinca alkaloid treatments. Both patients treated with vinca alkaloids for severe hemolysis responded. Two patients received oral cyclosporin for refractory ITP; one had a transient response. Splenectomy was not included in our regimen. Two patients underwent splenectomy prior to their referral; both required further therapy. In addition, review of the clinical courses of our patients indicate that the DAT may be a prognostic factor for the chronicity of ITP. CONCLUSION: In view of the high relapse rates after splenectomy and the encouraging results of our pilot study, we suggest that medical treatment with combination agents (IVIG, steroids, vinca alkaloids, androgens and possibly cyclosporin) may provide a useful therapeutic approach to patients with Evans syndrome.