Owaidah Tarek, Momen Abdulkareem Al, Alzahrani Hazzaa, Almusa Abdulrahman, Alkasim Fawaz, Tarawah Ahmed, Nouno Randa Al, Batniji Fatima Al, Alothman Fahad, Alomari Ali, Abu-Herbish Saud, Abu-Riash Mahmoud, Siddiqui Khawar, Ahmed Mansor, Mohamed S Y, Saleh Mahasen
aDepartment of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center bCenter of Excellence in Thrombosis and Hemostasis, King Saud University cOncology Center dDepartment of Pediatric Hematology, King Faisal Specialist Hospital and Research Center eDepartment of Pediatric Hematology, Ministry of Health, Riyadh fDepartment of Pediatric Hematology, Ministry of Health, Medina gDepartment of Pediatric Hematology, Military Hospital hDepartment of Pediatric Hematology, Security Force Hospital iDepartment of Pediatric Hematology, National Guard Hospital jDepartment of Oncology, Security Forces Hospital, Riyadh, Saudi Arabia.
Medicine (Baltimore). 2017 Jan;96(2):e5456. doi: 10.1097/MD.0000000000005456.
Hemophilia A and B are X-linked diseases that predominantly affect male patients. Patients can develop coagulation factor inhibitors, which exponentially increases the treatment cost. However, the prevalence of factor VIII and IX inhibitors in Saudi Arabia is unclear.This study aimed to determine the Saudi prevalence of factor VIII and IX inhibitors.This 4-year, 7-center, cross-sectional study evaluated the Saudi prevalences of hemophilia A and B. We collected the patients' clinical data, evaluated their disease, and tested for factor inhibitors.We included 202 patients with hemophilia (median age at diagnosis: 0.13 years, range: birth-34.8 years). The patients included 198 male patients (98%), 148 patients with hemophilia A (73.3%), and 54 patients with hemophilia B (26.7%). The patients exhibited severe factor VIII activity (<1%; 121 patients; 5.2%), moderate activity (1-5%; 7 patients; 4.9%), and mild activity (14 patients; 9.9%). Among the patients with care-related data, most patients were treated for episodic bleeding (76.8%) or received prophylaxis (22.6%); 1 patient received both treatments. Among the patients with source-related data, the factor replacements were derived from plasma (48.4%), recombinant concentrates (22.9%), both sources (14.6%), or fresh frozen plasma (14.1%). Factor VIII inhibitors were observed in 43 (29.3%) of the 147 patients, and only 1 of the 54 patients developed factor IX inhibitors. Most patients who developed inhibitors had severe hemophilia (40/44; 90.9%), and inhibitors were also common among patients who received recombinant products (14/43; 32.6%).The Saudi prevalence of factor inhibitors was similar to those among other ethnic populations.
甲型血友病和乙型血友病是主要影响男性患者的X连锁疾病。患者可能会产生凝血因子抑制剂，这会使治疗成本呈指数级增加。然而，沙特阿拉伯VIII因子和IX因子抑制剂的患病率尚不清楚。本研究旨在确定沙特阿拉伯VIII因子和IX因子抑制剂的患病率。这项为期4年、涉及7个中心的横断面研究评估了沙特阿拉伯甲型血友病和乙型血友病的患病率。我们收集了患者的临床数据，评估了他们的病情，并检测了因子抑制剂。我们纳入了202例血友病患者（诊断时的中位年龄：0.13岁，范围：出生至34.8岁）。患者包括198例男性患者（98%）、148例甲型血友病患者（73.3%）和54例乙型血友病患者（26.7%）。患者表现出严重的VIII因子活性（<1%；121例患者；5.2%）、中度活性（1-5%；7例患者；4.9%）和轻度活性（14例患者；9.9%）。在有护理相关数据的患者中，大多数患者接受了间歇性出血治疗（76.8%）或接受了预防治疗（22.6%）；1例患者接受了两种治疗。在有来源相关数据的患者中，因子替代物来自血浆（48.4%）、重组浓缩物（22.9%）、两种来源（14.6%）或新鲜冰冻血浆（14.1%）。在147例患者中有43例（29.3%）观察到VIII因子抑制剂，54例患者中只有1例出现IX因子抑制剂。大多数产生抑制剂的患者患有严重血友病（40/44；90.9%），并且抑制剂在接受重组产品治疗的患者中也很常见（14/43；32.6%）。沙特阿拉伯因子抑制剂的患病率与其他种族人群相似。
