Urban Christian, Lackner Herwig, Sovinz Petra, Benesch Martin, Schwinger Wolfgang, Dornbusch Hans Jürgen, Moser Andrea
Division of Pediatric Hematology/Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria.
Eur J Haematol. 2006 Jun;76(6):526-30. doi: 10.1111/j.0902-4441.2006.t01-1-EJH2549.x. Epub 2006 Mar 9.
Evans syndrome is an autoimmunopathy characterized by thrombocytopenia and autoimmune hemolytic anemia with poor response to immunosuppression. A 2-yr-old boy with Evans syndrome showed only short-lasting responses to immunosuppressive treatment including double autologous peripheral stem cell transplantation (SCT). Intracranial bleeding required emergency splenectomy and external ventricular drainage. Unrelated umbilical cord blood was given following conditioning with busulfan, thiotepa, etoposide and antithymocyte globulin. One year after SCT the patient shows stable blood counts without immunosuppression. This is the first child reported with Evans syndrome successfully treated by means of unrelated cord blood transplantation.
伊文氏综合征是一种自身免疫病，其特征为血小板减少和自身免疫性溶血性贫血，对免疫抑制治疗反应不佳。一名患有伊文氏综合征的2岁男孩对包括双重自体外周干细胞移植（SCT）在内的免疫抑制治疗仅表现出短暂反应。颅内出血需要紧急脾切除术和脑室外引流。在用白消安、噻替派、依托泊苷和抗胸腺细胞球蛋白进行预处理后，给予了无关供者脐带血。干细胞移植一年后，患者在未进行免疫抑制的情况下血细胞计数稳定。这是首例报道的通过无关供者脐带血移植成功治疗伊文氏综合征的儿童病例。
