Dorgalaleh Akbar, Dadashizadeh Ghazaleh, Bamedi Taregh
a Department of Hematology and Blood Transfusion, School of Allied Medical Sciences , Iran University of Medical Sciences , Tehran , Iran.
b Department of Hematology, School of Medicine , Mashhad University of Medical Sciences , Mashhad , Iran.
Hematology. 2016 Jun;21(5):300-10. doi: 10.1080/10245332.2015.1125080. Epub 2016 Feb 24.
Hemophilia A (HA) and B (HB) are common bleeding disorders, Iran having the ninth largest such population in the world. A considerable number of studies have been performed on different aspects of their disorder.
The aim of the study was to gather all obtainable data about Iranian patients with HA and HB, including molecular studies, clinical presentations and treatment, and development and management of patients with inhibitor, to help better understand the disease and its management in other parts of the world.
For this review study, we searched MEDLINE and Scientific Information Database for English and Persian sources until 2015.
There are 5369 patients with HA and HB in Iran among which 4438 patients have HA. About one-fifth of HA patients' genes were analyzed and their underlying defects detected. Hemarthrosis, epistaxis, ecchymosis, and post-dental extraction bleeding are the most common clinical presentations. Bleeding was mainly managed by on-demand replacement therapy with factor VIII/factor IX (FVIII/FIX) concentrates or cryoprecipitate in HA, and fresh frozen plasma in HB in the absence of factor concentrate. Mean per capita for FVIII in HA patients is 1.56 IU, which is higher than the global per capita mean. However, mean per capita for FIX (0.24 IU) is lower than the global mean but highest among eastern Mediterranean countries. Replacement with plasma-derived components has led to infection in a large number of patients as well as inhibitor development against exogenous infusion of coagulation factors. According to a World Federation of Hemophilia survey, 223 HA and 6 HB patients in Iran have developed inhibitor and have been mainly managed by recombinant FVII (rFVIIa) and activated prothrombin-complex concentrate.
Although this study was performed in Iranian patients, the large number thereof gives confidence that the results can be used more widely for other countries, especially in the developing world.
甲型血友病（HA）和乙型血友病（HB）是常见的出血性疾病，伊朗是世界上此类患者人数第九多的国家。针对其疾病的不同方面已经开展了大量研究。
本研究的目的是收集有关伊朗HA和HB患者的所有可获取数据，包括分子研究、临床表现与治疗，以及抑制剂患者的发展与管理情况，以帮助更好地了解该疾病及其在世界其他地区的管理方法。
对于本综述研究，我们检索了MEDLINE和科学信息数据库，以查找截至2015年的英文和波斯文资料来源。
伊朗有5369例HA和HB患者，其中4438例为HA患者。约五分之一的HA患者基因得到分析，并检测出其潜在缺陷。关节积血、鼻出血、瘀斑和拔牙后出血是最常见的临床表现。出血主要通过按需使用凝血因子VIII/凝血因子IX（FVIII/FIX）浓缩物或冷沉淀进行替代治疗，在HA中如此，在HB中若没有凝血因子浓缩物则使用新鲜冰冻血浆。HA患者的FVIII人均用量为1.56 IU，高于全球人均用量。然而，FIX的人均用量（0.24 IU）低于全球平均水平，但在东地中海国家中是最高的。使用血浆源性成分进行替代治疗导致大量患者感染，以及针对外源性凝血因子输注产生抑制剂。根据世界血友病联盟的一项调查，伊朗有223例HA患者和6例HB患者产生了抑制剂，主要通过重组FVII（rFVIIa）和活化凝血酶原复合物浓缩物进行管理。
尽管本研究是在伊朗患者中进行的，但患者数量众多，这使我们相信研究结果可更广泛地应用于其他国家，尤其是发展中国家。
