摘要
免疫介导血细胞减少症是常见的非骨髓造血异常所致的血细胞减少性疾病,在一线治疗无效情况下,患者生存质量大幅降低。雷帕霉素具有明确的抑制mTOR靶蛋白机制,可以上调调节性T细胞及其诱导特定细胞凋亡,通过调节淋巴细胞亚群从而对各类免疫介导血细胞减少治疗方面取得一定疗效。因此,本文就雷帕霉素治疗免疫性血小板减少症(immune thrombocytopenia,ITP)、自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)、获得性再生障碍性贫血及自身免疫性淋巴细胞增生综合症(autoimmune lymphoproliferative syndrome,ALPS)等免疫相关血细胞减少症的作用机制及临床应用进行综述。
Immune-mediated hemocytopenia is a common cytopenic diseases without bone marrow hematopoietic abnormalities,the patient' s quality of life is significantly reduced when first - line treatments are ineffective.Rapamycin,possesses a clear mechanism of targeting mTOR protein,can upregulate regulatory T cells and induces apoptosis of specific cells,by regulating the lymphocyte subsets,so as to treat various types of immune - mediated hemocytopenia with a certain therapeutic effect.In this reviews,the action mechanism and clinical application of rapamycin in immune thrombocytopenia( ITP) ,autoimmune hemolytic anemia( AIHA) ,acquired aplastic anemia and autoimmune lymphoproliferative syndrome( ALPS) etc.are summarized.
作者
谷昊
吴润晖
GU Hao;WU Run-Hui(Department of Hematology and Oncology,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China)
出处
《中国实验血液学杂志》
CAS
CSCD
北大核心
2018年第6期1836-1840,共5页
Journal of Experimental Hematology
