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。

IF 0.6 Q4 HEMATOLOGY Asian Journal of Transfusion Science Pub Date : 2022-07-01 Epub Date: 2022-09-28 DOI:10.4103/ajts.ajts_121_21
Ashwinkumar Vaidya, Isha Polavarapu, Ravindra Attur Prabhu, Ganesh Mohan, Shamee Shastry, P A Prethika
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引用次数: 1

摘要

非典型溶血性尿毒症综合征(aHUS)是一种罕见的危及生命的疾病,死亡率和发病率很高。aHUS的发病率约为每年每1000000人中有1或2例。病因可以是家族性的,也可以是散发性的。aHUS的发病机制涉及替代补体途径的失调,补体基因的易感突变。aHUS预后不良,临床病程逐渐或复发(30%-86%)。这种疾病可能出现在任何年龄,但主要发生在儿童和年轻人身上。治疗性血浆置换(TPE)是aHUS的主要治疗方式之一。本报告介绍了级联血浆置换术在复发性aHUS患者中的应用及其优于TPE的优势。级联血浆置换后抗H因子抗体水平下降73%。
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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.

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CiteScore
0.90
自引率
0.00%
发文量
56
审稿时长
44 weeks
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