Novie Amelia Chozie, Djajadiman Gatot, Bambang Sudarmanto, Susi Susanah, Rini Purnamasari, Pudjo Hagung Widjajanto, Susanto Nugroho, Olga Rasiyanti, Dian Puspitasari, Muhammad Riza, Maria Christina Shanty Larasati, Sri Suryo Adiyanti, Made Citra Saraswati, Fitri Primacakti
Background Factor VIII (FVIII) inhibitor diagnosis and surveillance in Indonesia are challenging owing to geographic conditions and the lack of laboratory facilities nationwide for inhibitor assays. This study aimed to determine the prevalence of FVIII inhibitors in children diagnosed with hemophilia A (HA) in Indonesia. Methods A cross-sectional study was conducted in 12 hospitals in eight provinces of Indonesia between 2020 and 2021. Factor VIII inhibitor screening was performed in a central hemostasis laboratory for all children with HA (≤18 yr) who had received a minimum of 10 exposure days to clotting factor concentrates. The FVIII inhibitor titer was determined using the Bethesda assay. Results Children (388) were enrolled in this study, including 219 (56.4%), 131 (33.8%), and 38 (9.4%) with severe, moderate, and mild HA, respectively. The prevalence of children who developed FVIII inhibitors was 37 out of 388 (9.6%). Factor VIII inhibitors were found in 25/219 (11.4%) severe, 11/131 (8.3%) moderate, and 1/38 (2.6%) children with mild HA. Thirteen children had low-titer inhibitors and 24 had high-titer inhibitors, with a median of 9.44 (1.48‒412.0) Bethesda Units. Among 13 children with low-titer inhibitors, eight underwent a confirmation test, of which five tested negative and were classified as transient. A significant difference in annual joint bleeding rate was found between patients with low and high inhibitor titers and those without inhibitors (P＜0.001). Conclusion Factor VIII inhibitor prevalence in Indonesia was relatively low. However, the risk factors that may contribute to FVIII inhibitor development among Indonesian patients require further study. © 2022 Korean Society of Hematology.
Department of Child Health, Dr. Cipto Mangunkusumo Hospital, Faculty of Medicine, Universitas Indonesia, Jakarta, Indonesia; Department of Child Health, Dr. Kariadi Hospital, Faculty of Medicine, Universitas Diponegoro, Semarang, Indonesia; Department of Child Health, Dr. Hasan Sadikin General Hospital, Bandung, Faculty of Medicine, Universitas Padjajaran, West Java, Indonesia; Department of Child Health, Tangerang General Hospital, Banten, Indonesia; Department of Child Health, Dr. Sardjito Hospital, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta, Indonesia; Department of Child Health, Dr. Saiful Anwar Hospital, Faculty of Medicine, Universitas Brawijaya, Malang, Indonesia; Department of Child Health, H. Adam Malik General Hospital, Faculty of Medicine, Universitas Sumatera Utara, Medan, Indonesia; Department of Child Health, Dr. Moh. Hoesin General Hospital, Faculty of Medicine, Universitas Sriwijaya, Palembang, Indonesia; Department of Child Health, Dr. Moewardi Hospital, Faculty of Medicine, Universitas Sebelas Maret, Surakarta, Indonesia; Department of Child Health, Dr. Soetomo General Hospital, Faculty of Medicine, Universitas Airlangga, Surabaya, Indonesia; Department of Clinical Pathology, Dr. Cipto Mangunkusumo Hospital, Faculty of Medicine, Universitas Indonesia, Jakarta, Indonesia
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