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FVIII inhibitor surveillance in children with hemophilia A in Indonesia: a report from the Indonesian Pediatric Hematology-Oncology Working Group

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 hemophil...

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Autores principales: Chozie, Novie Amelia, Gatot, Djajadiman, Sudarmanto, Bambang, Susanah, Susi, Purnamasari, Rini, Widjajanto, Pudjo Hagung, Nugroho, Susanto, Rasiyanti, Olga, Puspitasari, Dian, Riza, Muhammad, Larasati, Maria Christina Shanty, Adiyanti, Sri Suryo, Saraswati, Made Citra, Primacakti, Fitri
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Korean Society of Hematology; Korean Society of Blood and Marrow Transplantation; Korean Society of Pediatric Hematology-Oncology; Korean Society on Thrombosis and Hemostasis 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9812731/
https://www.ncbi.nlm.nih.gov/pubmed/36535639
http://dx.doi.org/10.5045/br.2022.2022153
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author Chozie, Novie Amelia
Gatot, Djajadiman
Sudarmanto, Bambang
Susanah, Susi
Purnamasari, Rini
Widjajanto, Pudjo Hagung
Nugroho, Susanto
Rasiyanti, Olga
Puspitasari, Dian
Riza, Muhammad
Larasati, Maria Christina Shanty
Adiyanti, Sri Suryo
Saraswati, Made Citra
Primacakti, Fitri
author_facet Chozie, Novie Amelia
Gatot, Djajadiman
Sudarmanto, Bambang
Susanah, Susi
Purnamasari, Rini
Widjajanto, Pudjo Hagung
Nugroho, Susanto
Rasiyanti, Olga
Puspitasari, Dian
Riza, Muhammad
Larasati, Maria Christina Shanty
Adiyanti, Sri Suryo
Saraswati, Made Citra
Primacakti, Fitri
author_sort Chozie, Novie Amelia
collection PubMed
description 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.
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spelling pubmed-98127312023-01-30 FVIII inhibitor surveillance in children with hemophilia A in Indonesia: a report from the Indonesian Pediatric Hematology-Oncology Working Group Chozie, Novie Amelia Gatot, Djajadiman Sudarmanto, Bambang Susanah, Susi Purnamasari, Rini Widjajanto, Pudjo Hagung Nugroho, Susanto Rasiyanti, Olga Puspitasari, Dian Riza, Muhammad Larasati, Maria Christina Shanty Adiyanti, Sri Suryo Saraswati, Made Citra Primacakti, Fitri Blood Res Original Article 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. Korean Society of Hematology; Korean Society of Blood and Marrow Transplantation; Korean Society of Pediatric Hematology-Oncology; Korean Society on Thrombosis and Hemostasis 2022-12-31 2022-12-31 /pmc/articles/PMC9812731/ /pubmed/36535639 http://dx.doi.org/10.5045/br.2022.2022153 Text en © 2022 Korean Society of Hematology https://creativecommons.org/licenses/by-nc/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0 (https://creativecommons.org/licenses/by-nc/4.0/) ) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Original Article
Chozie, Novie Amelia
Gatot, Djajadiman
Sudarmanto, Bambang
Susanah, Susi
Purnamasari, Rini
Widjajanto, Pudjo Hagung
Nugroho, Susanto
Rasiyanti, Olga
Puspitasari, Dian
Riza, Muhammad
Larasati, Maria Christina Shanty
Adiyanti, Sri Suryo
Saraswati, Made Citra
Primacakti, Fitri
FVIII inhibitor surveillance in children with hemophilia A in Indonesia: a report from the Indonesian Pediatric Hematology-Oncology Working Group
title FVIII inhibitor surveillance in children with hemophilia A in Indonesia: a report from the Indonesian Pediatric Hematology-Oncology Working Group
title_full FVIII inhibitor surveillance in children with hemophilia A in Indonesia: a report from the Indonesian Pediatric Hematology-Oncology Working Group
title_fullStr FVIII inhibitor surveillance in children with hemophilia A in Indonesia: a report from the Indonesian Pediatric Hematology-Oncology Working Group
title_full_unstemmed FVIII inhibitor surveillance in children with hemophilia A in Indonesia: a report from the Indonesian Pediatric Hematology-Oncology Working Group
title_short FVIII inhibitor surveillance in children with hemophilia A in Indonesia: a report from the Indonesian Pediatric Hematology-Oncology Working Group
title_sort fviii inhibitor surveillance in children with hemophilia a in indonesia: a report from the indonesian pediatric hematology-oncology working group
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9812731/
https://www.ncbi.nlm.nih.gov/pubmed/36535639
http://dx.doi.org/10.5045/br.2022.2022153
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