Santiago-Ríos Karen J, Reyes Omayra, Cruz Alexis, Rodríguez-Pabón Nydia, Cáceres William
From the Hematology-Oncology Section, Department of Medicine, VA Caribbean Healthcare System, 10 Calle Casia, San Juan, Puerto Rico 00921.
Bol Asoc Med P R. 2010 Apr-Jun;102(2):33-6.
Evans syndrome is an autoimmune disorder characterized by the simultaneous or sequential development of autoimmune hemolytic anemia and immune thrombocytopenia. It may be primary (idiopathic, or associated with other diseases. First line therapy is immunosupression. A second line therapy includes danazol and splenectomy. Rituximab was approved by the Federal Drug Administration since 1998 for the treatment of lymphomas. We report a 46-year-old-male Hispanic with Evans syndrome. He presented with severe life threatening autoimmune hemolytic anemia and subsequently developed autoimmune thrombocytopenia. After treatment with steroids and rituximab he remains in remission. This case report supports the use of rituximab in an adult patient with Evans syndrome.
伊文氏综合征是一种自身免疫性疾病,其特征为自身免疫性溶血性贫血和免疫性血小板减少症同时或相继出现。它可能是原发性的(特发性的,或与其他疾病相关)。一线治疗是免疫抑制。二线治疗包括达那唑和脾切除术。利妥昔单抗自1998年起被美国食品药品监督管理局批准用于治疗淋巴瘤。我们报告一例46岁的西班牙裔男性伊文氏综合征患者。他最初表现为严重的、危及生命的自身免疫性溶血性贫血,随后发展为自身免疫性血小板减少症。在接受类固醇和利妥昔单抗治疗后,他仍处于缓解状态。本病例报告支持利妥昔单抗在成年伊文氏综合征患者中的应用。