Ashwinkumar Vaidya, Isha Polavarapu, Ravindra Attur Prabhu, Ganesh Mohan, Shamee Shastry, P A Prethika
{"title":"级联血浆置换与常规治疗性血浆置换治疗复发性非典型溶血性尿毒症综合征的疗效比较:一例报告。","authors":"Ashwinkumar Vaidya, Isha Polavarapu, Ravindra Attur Prabhu, Ganesh Mohan, Shamee Shastry, P A Prethika","doi":"10.4103/ajts.ajts_121_21","DOIUrl":null,"url":null,"abstract":"<p><p>Atypical hemolytic uremic syndrome (aHUS) is a rare and life-threatening disease that is associated with high mortality and morbidity. The incidence of aHUS is about 1 or 2 cases per 1,000,000 per year. Etiology can be either familial or sporadic. The pathogenesis of aHUS involves dysregulation of the alternative complement pathway, with predisposing mutations in complement genes. aHUS has a poor prognosis and a gradual or a relapsing (30%-86%) clinical course. The disease may present at any age but is mostly seen in children and young adults. Therapeutic plasma exchange (TPE) is one of the primary modalities of treatment in aHUS. This report presents the utilization of cascade plasmapheresis and its advantages over TPE in a patient with relapsed aHUS. There was a 73% decrement in antifactor H antibody levels following cascade plasmapheresis.</p>","PeriodicalId":42296,"journal":{"name":"Asian Journal of Transfusion Science","volume":"16 2","pages":"283-286"},"PeriodicalIF":0.6000,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/15/d7/AJTS-16-283.PMC9855224.pdf","citationCount":"1","resultStr":"{\"title\":\"Efficacy of cascade plasmapheresis in comparison with conventional therapeutic plasma exchange for relapsed atypical hemolytic uremic syndrome: A case report.\",\"authors\":\"Ashwinkumar Vaidya, Isha Polavarapu, Ravindra Attur Prabhu, Ganesh Mohan, Shamee Shastry, P A Prethika\",\"doi\":\"10.4103/ajts.ajts_121_21\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Atypical hemolytic uremic syndrome (aHUS) is a rare and life-threatening disease that is associated with high mortality and morbidity. The incidence of aHUS is about 1 or 2 cases per 1,000,000 per year. Etiology can be either familial or sporadic. The pathogenesis of aHUS involves dysregulation of the alternative complement pathway, with predisposing mutations in complement genes. aHUS has a poor prognosis and a gradual or a relapsing (30%-86%) clinical course. The disease may present at any age but is mostly seen in children and young adults. Therapeutic plasma exchange (TPE) is one of the primary modalities of treatment in aHUS. This report presents the utilization of cascade plasmapheresis and its advantages over TPE in a patient with relapsed aHUS. There was a 73% decrement in antifactor H antibody levels following cascade plasmapheresis.</p>\",\"PeriodicalId\":42296,\"journal\":{\"name\":\"Asian Journal of Transfusion Science\",\"volume\":\"16 2\",\"pages\":\"283-286\"},\"PeriodicalIF\":0.6000,\"publicationDate\":\"2022-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/15/d7/AJTS-16-283.PMC9855224.pdf\",\"citationCount\":\"1\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Asian Journal of Transfusion Science\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/ajts.ajts_121_21\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2022/9/28 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q4\",\"JCRName\":\"HEMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Asian Journal of Transfusion Science","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/ajts.ajts_121_21","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2022/9/28 0:00:00","PubModel":"Epub","JCR":"Q4","JCRName":"HEMATOLOGY","Score":null,"Total":0}
Efficacy of cascade plasmapheresis in comparison with conventional therapeutic plasma exchange for relapsed atypical hemolytic uremic syndrome: A case report.
Atypical hemolytic uremic syndrome (aHUS) is a rare and life-threatening disease that is associated with high mortality and morbidity. The incidence of aHUS is about 1 or 2 cases per 1,000,000 per year. Etiology can be either familial or sporadic. The pathogenesis of aHUS involves dysregulation of the alternative complement pathway, with predisposing mutations in complement genes. aHUS has a poor prognosis and a gradual or a relapsing (30%-86%) clinical course. The disease may present at any age but is mostly seen in children and young adults. Therapeutic plasma exchange (TPE) is one of the primary modalities of treatment in aHUS. This report presents the utilization of cascade plasmapheresis and its advantages over TPE in a patient with relapsed aHUS. There was a 73% decrement in antifactor H antibody levels following cascade plasmapheresis.