Taresh A K, Hassan M K
Department of Pediatrics, Al-Muthana Maternity and Children's Hospital, Al-Muthana, Iraq.
Department of Pediatrics, College of Medicine, University of Basra; Center for Hereditary Blood Disease/Basra Health Directorate, Basra, Iraq.
Niger J Clin Pract. 2019 Mar;22(3):416-421. doi: 10.4103/njcp.njcp_388_18.
Inhibitor formation is a major complication of hemophilia treatment because it interferes with the clinical response to factor replacement and causes significant morbidity. This cross-sectional study was conducted to assess the presence and frequency of inhibitors among registered person with hemophilia and to identify risk factors associated with inhibitor development.
A total of 143 hemophilics, 118 with hemophilia A (HA) and 25 with hemophilia B (HB), were enrolled for the study. Participant's clinical data were obtained through patient's medical records. Factor VIII and IX levels and the presence of inhibitors were assessed using a fully automated coagulometer. From the results of a Bethesda assay, patients were divided into those with high titers (≥5 BU) and those with low titers (<5 BU).
The patient's age ranged from 1 to 67 years with median of 13.8 years. Inhibitors were detected in 18.6% and none of HA and HB patients, respectively. Of the 22 patients with HA and inhibitors, 18 (82%) had high titer inhibitors. The frequency of inhibitors was significantly higher among patients with severe hemophilia, a history of early exposure (≤3 months) to factor VIII concentrate, and family histories of autoimmune disease and immune system challenges (P < 0.05). The independent risk factors associated with inhibitor development were severe hemophilia (95% CIs = 1.02-55.6, OR = 7.5) and immune system challenges (95% CIs = 1.14-5.99, OR = 2.6).
Inhibitors were common among HA patients, and both severe HA and immune system challenges (surgery and trauma) are independent risk factors for inhibitor development.
抑制剂的形成是血友病治疗的主要并发症，因为它会干扰对凝血因子替代治疗的临床反应，并导致严重的发病率。本横断面研究旨在评估登记的血友病患者中抑制剂的存在情况和频率，并确定与抑制剂形成相关的危险因素。
本研究共纳入143例血友病患者，其中118例为甲型血友病（HA），25例为乙型血友病（HB）。通过患者病历获取参与者的临床数据。使用全自动凝血仪评估凝血因子VIII和IX水平以及抑制剂的存在情况。根据贝塞斯达检测结果，将患者分为高滴度（≥5 BU）和低滴度（<5 BU）两组。
患者年龄范围为1至67岁，中位数为13.8岁。分别在18.6%的HA患者和无HB患者中检测到抑制剂。在22例患有HA且有抑制剂的患者中，18例（82%）为高滴度抑制剂。在重度血友病患者、有早期（≤3个月）接触凝血因子VIII浓缩物史的患者以及有自身免疫性疾病和免疫系统刺激家族史的患者中，抑制剂的频率显著更高（P < 0.05）。与抑制剂形成相关的独立危险因素为重度血友病（95%置信区间 = 1.02 - 55.6，比值比 = 7.5）和免疫系统刺激（95%置信区间 = 1.14 - 5.99，比值比 = 2.6）。
抑制剂在HA患者中很常见，重度HA和免疫系统刺激（手术和创伤）都是抑制剂形成的独立危险因素。
