Abstract
Factor VIII replacement is the mainstay of treatment in hemophilia A but may lead to the development of inhibitors. While a vexing clinical problem, some observations suggest that the presence of inhibitors may not necessarily portend a higher bleeding risk. Our aim was to assess the prevalence and clinicopathological correlates of inhibitors in a well characterized cohort of Indian patients with HA patients. We retrospectively reviewed the clinical details and laboratory findings of consecutive hemophilia A patients attending a north-Indian tertiary-care center from 2010 to 2020. Among 592 patients with HA, inhibitors were detected in 35 patients (5.9%). Prevalence of inhibitors in moderate and severe hemophilia was 4.2% and 6.7%, respectively. Most patients with inhibitors had history of transfusion with factor VIII alone (54.3%) or a combination of factor VIII concentrate and other blood-products (42.9%). Intracranial bleed was significantly more frequent in patients with inhibitors compared to those without inhibitors (20% vs. 4.1%; p-0.001). Time dependent and immediately acting inhibitors were seen in 60% and 40% patients, respectively. High-titre (> 5 BU) and low-titre inhibitors (< 5 BU) were detected in 28 (80%) and 7 (20%) patients, respectively. Prevalence of inhibitors in our cohort was 5.9% and most had high-titre, time dependent inhibitors. These patients may have a higher risk of intracranial bleeding.
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DR, AJ and NK have designed the study, retrieved the data, and drafted the manuscript; CH and AJ performed the testing and helped in retrieving the data; RJ, DB, AT, ArJ, PM and JA performed the analytical analysis and helped in drafting of the manuscript. All authors approved the final manuscript.
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Ray, D., Kumar, N., Hans, C. et al. Inhibitor; An Uncommon But Vexing Challenge In North Indian Patients With Hemophilia A. Indian J Hematol Blood Transfus 38, 703–709 (2022). https://doi.org/10.1007/s12288-022-01539-9
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DOI: https://doi.org/10.1007/s12288-022-01539-9