{"title":"造血干细胞移植相关血栓性微血管病的发病机制、诊断和治疗现状","authors":"X. Liu","doi":"10.3760/CMA.J.ISSN.1673-419X.2019.06.001","DOIUrl":null,"url":null,"abstract":"Transplantation associated thrombotic microangiopathy (TA-TMA) is a significant complication after hematopoietic stem cell transplantation (HSCT), and may lead to a high risk of death. It is a multifactorial disorder focused on small vessel endothelial injury which can be trigged by varieties of mechanisms during HSCT. The complement system is found to play a role in the development of TA-TMA. Current laboratory diagnostic criteria are unsatisfactory and the latest diagnostic criterion includes activation of the complement system. But the pathological diagnosis remains to be the golden standard. Blocking the complement system with eculizumab may be one of the effective treatment for high-risk patients with TA-TMA. This article reviews the status on pathogenesis, diagnosis and treatment of TA-TMA. \n \n \nKey words: \nThrombotic microangiopathies; Hematopoietic stem cell transplantation; Endothelium, vascular; Complement system proteins; Transplantation associated thrombotic microangiopathy; Endothelial injury; Eculizumab","PeriodicalId":13774,"journal":{"name":"国际输血及血液学杂志","volume":"42 1","pages":"461-468"},"PeriodicalIF":0.0000,"publicationDate":"2019-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Status on pathogenesis, diagnosis and treatment of hematopoietic stem cell transplantation associated thrombotic microangiopathy\",\"authors\":\"X. Liu\",\"doi\":\"10.3760/CMA.J.ISSN.1673-419X.2019.06.001\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Transplantation associated thrombotic microangiopathy (TA-TMA) is a significant complication after hematopoietic stem cell transplantation (HSCT), and may lead to a high risk of death. It is a multifactorial disorder focused on small vessel endothelial injury which can be trigged by varieties of mechanisms during HSCT. The complement system is found to play a role in the development of TA-TMA. Current laboratory diagnostic criteria are unsatisfactory and the latest diagnostic criterion includes activation of the complement system. But the pathological diagnosis remains to be the golden standard. Blocking the complement system with eculizumab may be one of the effective treatment for high-risk patients with TA-TMA. This article reviews the status on pathogenesis, diagnosis and treatment of TA-TMA. \\n \\n \\nKey words: \\nThrombotic microangiopathies; Hematopoietic stem cell transplantation; Endothelium, vascular; Complement system proteins; Transplantation associated thrombotic microangiopathy; Endothelial injury; Eculizumab\",\"PeriodicalId\":13774,\"journal\":{\"name\":\"国际输血及血液学杂志\",\"volume\":\"42 1\",\"pages\":\"461-468\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2019-11-20\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"国际输血及血液学杂志\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.3760/CMA.J.ISSN.1673-419X.2019.06.001\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"国际输血及血液学杂志","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.3760/CMA.J.ISSN.1673-419X.2019.06.001","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Status on pathogenesis, diagnosis and treatment of hematopoietic stem cell transplantation associated thrombotic microangiopathy
Transplantation associated thrombotic microangiopathy (TA-TMA) is a significant complication after hematopoietic stem cell transplantation (HSCT), and may lead to a high risk of death. It is a multifactorial disorder focused on small vessel endothelial injury which can be trigged by varieties of mechanisms during HSCT. The complement system is found to play a role in the development of TA-TMA. Current laboratory diagnostic criteria are unsatisfactory and the latest diagnostic criterion includes activation of the complement system. But the pathological diagnosis remains to be the golden standard. Blocking the complement system with eculizumab may be one of the effective treatment for high-risk patients with TA-TMA. This article reviews the status on pathogenesis, diagnosis and treatment of TA-TMA.
Key words:
Thrombotic microangiopathies; Hematopoietic stem cell transplantation; Endothelium, vascular; Complement system proteins; Transplantation associated thrombotic microangiopathy; Endothelial injury; Eculizumab
期刊介绍:
The International Journal of Transfusion and Hematology was founded in September 1978. It is a comprehensive academic journal in the field of transfusion and hematology, supervised by the National Health Commission and co-sponsored by the Chinese Medical Association, West China Second Hospital of Sichuan University, and the Institute of Transfusion Medicine of the Chinese Academy of Medical Sciences. The journal is a comprehensive academic journal that combines the basic and clinical aspects of transfusion and hematology and is publicly distributed at home and abroad. The International Journal of Transfusion and Hematology mainly reports on the basic and clinical scientific research results and progress in the field of transfusion and hematology, new experiences, new methods, and new technologies in clinical diagnosis and treatment, introduces domestic and foreign research trends, conducts academic exchanges, and promotes the development of basic and clinical research in the field of transfusion and hematology.