Oyama Y, Papadopoulos E B, Miranda M, Traynor A E, Burt R K
Division of Immune Therapy and Autoimmune Diseases, Northwestern University Medical Center, Chicago, IL, USA.
Bone Marrow Transplant. 2001 Nov;28(9):903-5. doi: 10.1038/sj.bmt.1703237.
Evans syndrome is a rare disorder characterized by combined autoimmune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Standard treatments consist of transfusions, corticosteroids, splenectomy, IVIG, anabolic steroids, vincristine, alkylating agents, or cyclosporine. In a patient with refractory disease, an allogeneic hematopoietic stem cell transplant (HSCT) resulted in complete clinical and serologic remission for more than 30 months. Allogeneic HSCT may be the only current curative therapy for Evans syndrome but may also be complicated by significant toxicities.
伊文氏综合征是一种罕见的疾病,其特征为自身免疫性血小板减少症(ITP)和自身免疫性溶血性贫血(AIHA)同时存在。标准治疗方法包括输血、皮质类固醇、脾切除术、静脉注射免疫球蛋白、合成代谢类固醇、长春新碱、烷化剂或环孢素。对于一名难治性疾病患者,异基因造血干细胞移植(HSCT)导致临床和血清学完全缓解超过30个月。异基因HSCT可能是目前伊文氏综合征唯一的治愈性疗法,但也可能伴有严重的毒性反应。