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首页> 外文期刊>Egyptian Journal of Medical Human Genetics >Pathophysiology of bleeding diathesis in haemophilia-A: A sequential and critical appraisal of non-FVIII related haemostatic dysfunctions and their therapeutic implications
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Pathophysiology of bleeding diathesis in haemophilia-A: A sequential and critical appraisal of non-FVIII related haemostatic dysfunctions and their therapeutic implications

机译：A型血友病出血素质的病理生理学：对非FVIII相关的止血功能障碍的顺序和严格评估及其治疗意义

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Haemophilia-A is characterized by deficiency of FVIII, but the bleeding diathesis is not a mere reflection low FVIII activity. The pathophysiology of haemophilic bleeding diathesis is a complex interplay between defective procoagulant function and up-regulated fibrinolysis. Moreover, haemophilic bleeding diathesis is frequently compounded by treatment-related and infective complications such as FVIII inhibitors, hepatitis, HIV infection, non-steroidal anti-inflammatory drugs (NSAID) induced gastritis, and infective mucosal injuries such as H pylori gastritis and intestinal and urinary helminthiasis. Hence, pathophysiology of haemophilic bleeding is multi-factorial, encompassing both FVIII and non-FVIII haemostatic defects. Currently available literature on pathophysiologic roles of non-FVIII haemostatic defects in haemophilia is fragmented. This articles is aimed at providing a composite and comprehensive review of the roles of non-FVIII haemostatic defects and their therapeutic implications in haemophilic bleeding diathesis, which will enable a holistic approach towards clinical management of the bleeding diathesis. This is necessary because FVIII therapy alone maybe insufficient in managing complicated haemophilic bleeding unless compounding non-FVIII-related haemostatic dysfunctions and comorbidities are identified, targeted and treated. This will necessitate appropriate use of non-FVIII therapeutic modalities, which may include anti-fibrinolytic agents, FVIII by-passing agents, immune modulation, and anti-microbial agents. Lots of work has been done in the areas of non-FVIII agents and FVIII by-pass therapy in the management of haemophilia, but more research is needed to validate many of these targeted therapeutic techniques. Meanwhile, healthcare personnel must consider the roles of both FVIII and non-FVIII haemostatic defects when evaluating haemophilic bleeding diathesis for the purpose of choosing appropriate and optimal treatment options.
机译：甲型血友病的特征是缺乏FVIII，但出血素质并非仅仅是FVIII活性低的反映。血友病出血素质的病理生理学是促凝血功能缺陷与纤维蛋白溶解上调之间的复杂相互作用。此外，血友病出血的素质常常与治疗相关和感染性并发症（例如FVIII抑制剂，肝炎，HIV感染，非甾体抗炎药（NSAID）诱发的胃炎）以及感染性粘膜损伤（例如幽门螺杆菌，胃炎和肠胃疾病）加重尿蠕虫病。因此，血友病性出血的病理生理是多因素的，既包括FVIII止血缺陷，也包括非FVIII止血缺陷。目前关于非FVIII止血缺陷在血友病中的病理生理作用的文献不完整。本文旨在对非FVIII止血缺陷的作用及其在嗜血性出血性疾病中的治疗意义提供综合而全面的综述，这将为出血性疾病的临床管理提供一种整体方法。这是必要的，因为除非确定，靶向和治疗非FVIII相关的止血功能障碍和合并症，否则仅FVIII疗法可能不足以处理复杂的血友病出血。这将需要适当使用非FVIII治疗方式，包括抗纤溶剂，FVIII旁路剂，免疫调节剂和抗微生物剂。在血友病的治疗中，在非FVIII药物和FVIII旁路疗法领域已经做了大量工作，但是需要更多的研究来验证许多这些靶向治疗技术。同时，医护人员在评估血友病出血素质时，必须考虑FVIII和非FVIII止血缺陷的作用，以选择适当和最佳的治疗方案。

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