Murakhovskaya Irina
Department of Hematology and Oncology, Albert Einstein College of Medicine/Montefiore Medical Center, Bronx, NY 10467, USA.
J Clin Med. 2020 Dec 13;9(12):4034. doi: 10.3390/jcm9124034.
Autoimmune hemolytic anemia is a rare condition characterized by destruction of red blood cells with and without involvement of complement. It is associated with significant morbidity and mortality. In warm autoimmune hemolytic anemia, less than 50% of patients remain in long-term remission following initial steroid therapy and subsequent therapies are required. Cold agglutinin disease is a clonal hematologic disorder that requires therapy in the majority of patients and responds poorly to steroids and alkylators. Rituximab has a favorable toxicity profile and has demonstrated efficacy in autoimmune hemolytic anemia in first-line as well as relapsed settings. Rituximab is the preferred therapy for steroid refractory warm autoimmune hemolytic anemia (wAIHA) and as part of the first- and second-line treatment of cold agglutinin disease. This article reviews the mechanism of action of rituximab and the current literature on its role in the management of primary and secondary warm autoimmune hemolytic anemia and cold agglutinin disease.
自身免疫性溶血性贫血是一种罕见疾病，其特征为红细胞的破坏，可伴有或不伴有补体参与。它与显著的发病率和死亡率相关。在温抗体型自身免疫性溶血性贫血中，初始使用类固醇治疗后，不到50%的患者能长期缓解，因此需要后续治疗。冷凝集素病是一种克隆性血液系统疾病，大多数患者需要治疗，且对类固醇和烷化剂反应不佳。利妥昔单抗具有良好的毒性特征，已在一线治疗以及复发情况下的自身免疫性溶血性贫血中显示出疗效。利妥昔单抗是类固醇难治性温抗体型自身免疫性溶血性贫血（wAIHA）的首选治疗方法，也是冷凝集素病一线和二线治疗的一部分。本文综述了利妥昔单抗的作用机制以及当前关于其在原发性和继发性温抗体型自身免疫性溶血性贫血及冷凝集素病管理中作用的文献。
