The prevalence of factor VIII and IX inhibitors among Saudi patients with hemophilia Results from the Saudi national hemophilia screening program

被引:12
|
作者
Owaidah, Tarek [1 ,2 ]
Al Momen, Abdulkareem [2 ]
Alzahrani, Hazzaa [3 ]
Almusa, Abdulrahman [4 ]
Alkasim, Fawaz [5 ]
Tarawah, Ahmed [6 ]
Al Nouno, Randa [1 ]
Al Batniji, Fatima [7 ]
Alothman, Fahad [8 ]
Alomari, Ali [9 ]
Abu-Herbish, Saud [10 ]
Abu-Riash, Mahmoud [3 ]
Siddiqui, Khawar [4 ]
Ahmed, Mansor [1 ]
Mohamed, S. Y. [3 ]
Saleh, Mahasen [4 ]
机构
[1] King Faisal Specialist Hosp & Res Ctr, Dept Pathol & Lab Med, POB 3354, Riyadh 11211, Saudi Arabia
[2] King Saud Univ, Ctr Excellence Thrombosis & Hemostasis, Riyadh, Saudi Arabia
[3] King Faisal Specialist Hosp & Res Ctr, Oncol Ctr, Riyadh, Saudi Arabia
[4] King Faisal Specialist Hosp & Res Ctr, Dept Pediat Hematol, Riyadh, Saudi Arabia
[5] Minist Hlth, Dept Pediat Hematol, Riyadh, Saudi Arabia
[6] Minist Hlth, Dept Pediat Hematol, Medina, Saudi Arabia
[7] Mil Hosp, Dept Pediat Hematol, Riyadh, Saudi Arabia
[8] Secur Force Hosp, Dept Pediat Hematol, Riyadh, Saudi Arabia
[9] Natl Guard Hosp, Dept Pediat Hematol, Riyadh, Saudi Arabia
[10] Secur Forces Hosp, Dept Oncol, Riyadh, Saudi Arabia
关键词
factor inhibitors; factor IX; factor VIII; hemophilia; hemostasis; Saudi Arabia; RECOMBINANT FACTOR-VIII; ON-DEMAND TREATMENT; FVIII INHIBITORS; MOLECULAR-BASIS; RISK-FACTORS; EPIDEMIOLOGY; COAGULATION; SURVEILLANCE; PROPHYLAXIS; ANTIBODIES;
D O I
10.1097/MD.0000000000005456
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
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页数:7
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