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所有纤维蛋白原浓缩剂都一样吗?两种纤维蛋白原治疗方法对一名无纤维蛋白原血症患者及纤维蛋白原缺乏血浆模型的影响。一项临床与实验室病例报告。

Are all fibrinogen concentrates the same? The effects of two fibrinogen therapies in an afibrinogenemic patient and in a fibrinogen deficient plasma model. A clinical and laboratory case report.

作者信息

Goodarzi Soutiam, Abu-Hanna Jeries, Harper Sarah, Khan Dalia, Morrow Gael, Curry Nicola

机构信息

Oxford University Medical School, Medical Sciences Division, John Radcliffe Hospital, Oxford, United Kingdom.

Radcliffe Department of Medicine, Oxford University, Oxford, United Kingdom.

出版信息

Front Med (Lausanne). 2024 May 30;11:1391422. doi: 10.3389/fmed.2024.1391422. eCollection 2024.

DOI:10.3389/fmed.2024.1391422
PMID:38873197
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11169818/
Abstract

The choice of treatments for inherited, or acquired, fibrinogen deficient states is expanding and there are now several fibrinogen concentrate therapies commercially available. Patients with the rare inherited bleeding disorder, afibrinogenemia, commonly require life-long replacement therapy with fibrinogen concentrate to prevent hemorrhagic complications. Recent reports in the setting of acquired bleeding, namely trauma hemorrhage, have highlighted the potential importance of the different compositions of fibrinogen supplements, including cryoprecipitate and the various plasma- derived concentrates. Clot strength and the subsequent susceptibility of a clot to lysis is highly dependent on the amount of fibrinogen as well as its structural composition, the concentration of pro- and anti-coagulant factors, as well as fibrinolytic regulators, such as factor XIII (FXIII). This report details the effects of two commercially available fibrinogen concentrates (Riastap, CSL Behring and Fibryga, Octapharma) on important functional measures of clot formation and lysis in a patient with afibrinogenemia. Our report offers insights into the differential effects of these concentrates, at the clot level, according to the variable constituents of each product, thereby emphasizing that the choice of fibrinogen concentrate can influence the stability of a clot . Whether this alters clinical efficacy is yet to be understood.

摘要

对于遗传性或获得性纤维蛋白原缺乏症的治疗选择正在不断增加,目前有几种纤维蛋白原浓缩物疗法可供商业使用。患有罕见遗传性出血性疾病——无纤维蛋白原血症的患者通常需要终身使用纤维蛋白原浓缩物进行替代治疗,以预防出血并发症。最近关于获得性出血(即创伤性出血)的报道强调了纤维蛋白原补充剂不同成分(包括冷沉淀和各种血浆衍生浓缩物)的潜在重要性。血凝块强度以及随后血凝块对溶解的敏感性高度依赖于纤维蛋白原的量及其结构组成、促凝血和抗凝血因子的浓度以及纤维蛋白溶解调节剂,如因子 XIII(FXIII)。本报告详细介绍了两种市售纤维蛋白原浓缩物(Riastap,CSL Behring公司;Fibryga,奥曲肽公司)对一名无纤维蛋白原血症患者血凝块形成和溶解的重要功能指标的影响。我们的报告根据每种产品的可变成分,在血凝块水平上深入了解了这些浓缩物的不同作用,从而强调纤维蛋白原浓缩物的选择会影响血凝块的稳定性。这是否会改变临床疗效尚有待了解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9089/11169818/b193eeca7051/fmed-11-1391422-g0004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9089/11169818/fef131c2e1ea/fmed-11-1391422-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9089/11169818/702f9dc254a3/fmed-11-1391422-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9089/11169818/c400972bb441/fmed-11-1391422-g0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9089/11169818/b193eeca7051/fmed-11-1391422-g0004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9089/11169818/fef131c2e1ea/fmed-11-1391422-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9089/11169818/702f9dc254a3/fmed-11-1391422-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9089/11169818/c400972bb441/fmed-11-1391422-g0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9089/11169818/b193eeca7051/fmed-11-1391422-g0004.jpg

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