当前位置: X-MOL 学术Br. J. Haematol. › 论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
The role of anti-complement factor H antibodies in the development of atypical haemolytic uremic syndrome: a possible contribution to abnormality of platelet function.
British Journal of Haematology ( IF 6.5 ) Pub Date : 2019-12-26 , DOI: 10.1111/bjh.16297
Madoka Fujisawa 1 , Atsushi Yasumoto 2 , Hideki Kato 1 , Yuka Sugawara 1 , Yoko Yoshida 1 , Yutaka Yatomi 2 , Masaomi Nangaku 1
Affiliation  

Atypical haemolytic uremic syndrome (aHUS) is associated with complement system abnormality, such as production of complement factor H (CFH) autoantibodies. The growing evidence indicates complement overactivation on platelets is intimately involved in aHUS pathogenesis, besides endothelial injury. We here showed plasma from patients with anti-CFH antibodies induced aggregation of washed platelets, while purified anti-CFH antibodies suppressed aggregation. This suggested anti-CFH antibody itself suppressed thrombosis, while other plasma factor including complement factors could overactivate the platelets, leading to aggregation, which augmented the notion the state of complement activation influenced by anti-CFH antibodies is important in the aggregation of platelets in aHUS.

中文翻译:

抗补体因子H抗体在非典型溶血性尿毒症候群发展中的作用:可能是血小板功能异常的原因。

非典型溶血性尿毒症综合征(aHUS)与补体系统异常有关,例如补体因子H(CFH)自身抗体的产生。越来越多的证据表明,除了内皮损伤外,血小板补体过度活化还与aHUS发病机理密切相关。我们在这里显示了来自具有抗CFH抗体的患者的血浆诱导了洗涤血小板的聚集,而纯化的抗CFH抗体抑制了聚集。这表明抗CFH抗体本身可抑制血栓形成,而其他血浆因子(包括补体因子)则可能使血小板过度活化,从而导致聚集,这增加了抗CFH抗体影响的补体激活状态的观点对于aHUS中血小板的聚集很重要。 。
更新日期:2019-12-27
down
wechat
bug