a Haematology , Mashhad University of Medical sciences (MUMS) , Mashhad , Iran.
b Allergy Research Center, Ghaem hospital, School of Medicine , Mashhad University of Medical Sciences , Mashhad , Iran.
Expert Rev Hematol. 2016 Sep;9(9):903-13. doi: 10.1080/17474086.2016.1208554. Epub 2016 Aug 16.
Hemophilia A (HA) has an X-linked pattern of inheritance and is the most common of the hemorrhagic disorders. HA is caused by a decreased or deficiency of the functional clotting factor VIII (FVIII) and effects 1 in 5000-10,000 male births. The common treatment for hemophilia is replacement therapy by plasma-derived or recombinant FVIII. Approximately 20-30% of people with a severe type of HA develop an inhibitor and this phenomenon is the main challenge in the management of these patients. Genetic factors and environmental determinants contribute to inhibitor development. Here, the roles of various genetic and environmental factors such as the type of FVIII concentrate used, the number of exposure days, and peak treatment time will be discussed in detail. It seems this information is helpful for hematologists.
A literature review was done in January 2016 on PubMed and Scopus using the following keywords:' h(a)emophilia A & factor VIII inhibitor', 'h(a)emophilia A & factor VIII alloantibody', 'h(a)emophilia A & inhibitor'. There was no time limitation; however, there was an English language limitation placed on the articles selected. Expert commentary: Influential genetic and environmental factors in developing inhibitors have been discussed. Most of the risk factors are related to previously untreated patients with hemophili.
血友病 A（HA）呈 X 连锁遗传，是最常见的出血性疾病之一。HA 是由功能性凝血因子 VIII（FVIII）减少或缺乏引起的，影响每 5000-10000 名男性出生中的 1 名。HA 的常见治疗方法是通过血浆衍生或重组 FVIII 进行替代疗法。大约 20-30%的严重型 HA 患者会产生抑制剂，这种现象是这些患者治疗管理的主要挑战。遗传因素和环境决定因素有助于抑制剂的发展。在这里，将详细讨论各种遗传和环境因素的作用，如所使用的 FVIII 浓缩物的类型、暴露天数和峰值治疗时间。这些信息似乎对血液学家有帮助。
2016 年 1 月，在 PubMed 和 Scopus 上使用以下关键字进行了文献回顾：'h(a)emophilia A & factor VIII inhibitor'、'h(a)emophilia A & factor VIII alloantibody'、'h(a)emophilia A & inhibitor'。没有时间限制；但是，所选文章的语言限于英语。专家评论：已经讨论了产生抑制剂的有影响力的遗传和环境因素。大多数危险因素与未经治疗的血友病患者有关。
