title: The prevalence of factor VIII and IX inhibitors among Saudi patients with hemophilia: Results from the Saudi national hemophilia screening program.
abstract: 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.
authors.affiliation: 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.
authors.forename: Tarek
authors.initials: T
authors.lastname: Owaidah
authors.forename: Abdulkareem Al
authors.initials: AA
authors.lastname: Momen
authors.forename: Hazzaa
authors.initials: H
authors.lastname: Alzahrani
authors.forename: Abdulrahman
authors.initials: A
authors.lastname: Almusa
authors.forename: Fawaz
authors.initials: F
authors.lastname: Alkasim
authors.forename: Ahmed
authors.initials: A
authors.lastname: Tarawah
authors.forename: Randa Al
authors.initials: RA
authors.lastname: Nouno
authors.forename: Fatima Al
authors.initials: FA
authors.lastname: Batniji
authors.forename: Fahad
authors.initials: F
authors.lastname: Alothman
authors.forename: Ali
authors.initials: A
authors.lastname: Alomari
authors.forename: Saud
authors.initials: S
authors.lastname: Abu-Herbish
authors.forename: Mahmoud
authors.initials: M
authors.lastname: Abu-Riash
authors.forename: Khawar
authors.initials: K
authors.lastname: Siddiqui
authors.forename: Mansor
authors.initials: M
authors.lastname: Ahmed
authors.forename: S Y
authors.initials: SY
authors.lastname: Mohamed
authors.forename: Mahasen
authors.initials: M
authors.lastname: Saleh
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004351:Drug Resistance; D005164:Factor IX / Q000037:antagonists & inhibitors*; D005169:Factor VIII / Q000037:antagonists & inhibitors*; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000150:complications / Q000188:drug therapy*; D002836:Hemophilia B / Q000097:blood / Q000150:complications / Q000188:drug therapy*; D006526:Hepatitis C / Q000097:blood / Q000150:complications; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007592:Joint Diseases / Q000209:etiology; D008297:Male; D012529:Saudi Arabia; D055815:Young Adult
chemical_list: D005169:Factor VIII; D005164:Factor IX