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ORIGINAL ARTICLE
Year : 2019  |  Volume : 22  |  Issue : 3  |  Page : 416-421

Inhibitors among patients with hemophilia in Basra, Iraq – A single center experience


1 Department of Pediatrics, Al-Muthana Maternity and Children's Hospital, Al-Muthana, Iraq
2 Department of Pediatrics, College of Medicine, University of Basra; Center for Hereditary Blood Disease/Basra Health Directorate, Basra, Iraq

Correspondence Address:
Prof. M K Hassan
Department of Pediatrics, College of Medicine, University of Basra, Basra
Iraq
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njcp.njcp_388_18

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Introduction: 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. Patients and Methods: 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). Results: 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). Conclusion: Inhibitors were common among HA patients, and both severe HA and immune system challenges (surgery and trauma) are independent risk factors for inhibitor development.


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