Development Of Inhibitors In Hemophilia A Patients Receiving Different Treatment Options

Isma Imitiaz, Aiman Mahmood Minhas, None Ayesha Younus, None Usman Imtiaz, None Hareem Noor, None Ayisha Imran
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Abstract

Introduction: Hemophilia A is the most common inherited bleeding disorder in Pakistan. Apart from frequent bleeds and joint deformities, the formation of inhibitors is the major complication of hemophilia. Inhibitors are antibodies formed against the coagulation factor. The purpose of this study is to determine the incidence of inhibitor development in hemophilia A patient receiving different treatment modalities namely fresh frozen plasma (FFP)/cryoprecipitate, plasma derived FVIII concentrates and long-acting recombinant FVIII concentrates. Aims & Objectives: To assess the development of inhibitors in hemophilia A patients receiving treatment modalities. Place and Duration of Study: The study was conducted at Chughtai Institute of Pathology from November 2021 to November 2023. Material &Methods: A total of 75 registered patients of the Hemophilia Patient Welfare Society, Lahore and Sundas Foundation were initially screened for FVIII inhibitors. Those who were found negative for these inhibitors were included in the study and then divided into three equal groups of 25 patients each. One group received only FFP/cryoprecipitate for treatment, the other group received plasma derived FVIII concentrates and the last group received recombinant factor VIII concentrates. These patients were observed for two years i.e after 15-20 exposures and following this period their inhibitors screening was done again to establish the effect of different treatment modalities on inhibitor development. The data collected was entered and analyzed by SPSS-21, a p-value of ?0.05 was considered significant. Results: Our study showed that only 3 patients out of 25, receiving FFP as treatment modality developed inhibitors. Whereas, none of the patients receiving plasma derived FVIII concentrates and recombinant factor VIII concentrates developed inhibitors. The P value calculated is 0.044 which is significant. Conclusion: Plasma derived FVIII concentrates and recombinant FVIII concentrates are safer as compared to FFP/Cryoprecipitate and have lesser risk for inhibitor development.
接受不同治疗方案的A型血友病患者抑制剂的研究进展
A型血友病是巴基斯坦最常见的遗传性出血性疾病。除了频繁出血和关节畸形外,抑制剂的形成是血友病的主要并发症。抑制剂是针对凝血因子形成的抗体。本研究的目的是确定在接受不同治疗方式(新鲜冷冻血浆/冷沉淀、血浆衍生FVIII浓缩物和长效重组FVIII浓缩物)的血友病A患者中抑制剂发展的发生率。的目标是,目的:评估A型血友病患者接受治疗方式时抑制剂的发展。研究地点和时间:研究于2021年11月至2023年11月在中台病理研究所进行。材料和方法:对血友病患者福利协会、拉合尔和Sundas基金会的75名注册患者进行FVIII抑制剂的初步筛选。这些抑制剂呈阴性的患者被纳入研究,然后被分成三组,每组25名患者。其中一组只接受FFP/低温沉淀治疗,另一组接受血浆源性FVIII浓缩物治疗,最后一组接受重组因子VIII浓缩物治疗。这些患者被观察了两年,即在15-20次暴露后,在此期间再次进行抑制剂筛选,以确定不同治疗方式对抑制剂发展的影响。收集的数据采用SPSS-21进行录入和分析,p值为0.05为显著性。结果:我们的研究显示,25例接受FFP治疗的患者中只有3例出现了抑制剂。然而,接受血浆源性FVIII浓缩物和重组因子VIII浓缩物的患者没有出现抑制剂。计算得到的P值为0.044,具有显著性。结论:血浆衍生FVIII浓缩物和重组FVIII浓缩物与FFP/ cryop沉淀物相比更安全,抑制剂发展的风险更小。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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