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Management of haemophilia A with inhibitors: A regional cross‐talk
INTRODUCTION: The development of inhibitors with factor VIII (FVIII) replacement therapy is one of the most common and challenging complications of haemophilia A (HA) treatment, jeopardising treatment efficacy and predisposing patients to high risks of morbidity and mortality. The management of pati...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley and Sons Inc.
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9796719/ https://www.ncbi.nlm.nih.gov/pubmed/35868021 http://dx.doi.org/10.1111/hae.14638 |
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author | Peyvandi, Flora Kavakli, Kaan El‐Beshlawy, Amal Rangarajan, Savita |
author_facet | Peyvandi, Flora Kavakli, Kaan El‐Beshlawy, Amal Rangarajan, Savita |
author_sort | Peyvandi, Flora |
collection | PubMed |
description | INTRODUCTION: The development of inhibitors with factor VIII (FVIII) replacement therapy is one of the most common and challenging complications of haemophilia A (HA) treatment, jeopardising treatment efficacy and predisposing patients to high risks of morbidity and mortality. The management of patients with inhibitors is particularly challenging in countries where resources are limited. AIM: To provide a comprehensive summary of the management of HA with inhibitors while focusing on differences in practice between Western and non‐Western countries and how resource scarcity can impact HA management, leading to suboptimal outcomes in patients with inhibitors. METHODS: Summary of key evidence and regional expert opinion. RESULTS: We address, particularly, the diagnosis of and testing for inhibitors, as well as the epidemiology of inhibitors, including incidence, prevalence and disease burden. Secondly, we provide an overview of the current treatment landscape in HA with inhibitors regarding the eradication of inhibitors with immune tolerance induction and the treatment and prevention of bleeding with bypassing agents, non‐factor replacement agents and other experimental therapies. This is complemented with insights from the authors around the applicability of, and challenges associated with, such therapies in their settings of practice. CONCLUSIONS: We conclude by proposing some key steps towards bridging the gaps in the management of HA with inhibitors in resource‐limited countries, including: (1) the collection of quality data that can inform healthcare reforms and policies; (2) improving disease knowledge among healthcare practitioners and patients with the aim of standardising disease management across centres and (3) working towards promoting equal access to HA care and therapies for everyone. |
format | Online Article Text |
id | pubmed-9796719 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | John Wiley and Sons Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-97967192023-01-04 Management of haemophilia A with inhibitors: A regional cross‐talk Peyvandi, Flora Kavakli, Kaan El‐Beshlawy, Amal Rangarajan, Savita Haemophilia Reviews INTRODUCTION: The development of inhibitors with factor VIII (FVIII) replacement therapy is one of the most common and challenging complications of haemophilia A (HA) treatment, jeopardising treatment efficacy and predisposing patients to high risks of morbidity and mortality. The management of patients with inhibitors is particularly challenging in countries where resources are limited. AIM: To provide a comprehensive summary of the management of HA with inhibitors while focusing on differences in practice between Western and non‐Western countries and how resource scarcity can impact HA management, leading to suboptimal outcomes in patients with inhibitors. METHODS: Summary of key evidence and regional expert opinion. RESULTS: We address, particularly, the diagnosis of and testing for inhibitors, as well as the epidemiology of inhibitors, including incidence, prevalence and disease burden. Secondly, we provide an overview of the current treatment landscape in HA with inhibitors regarding the eradication of inhibitors with immune tolerance induction and the treatment and prevention of bleeding with bypassing agents, non‐factor replacement agents and other experimental therapies. This is complemented with insights from the authors around the applicability of, and challenges associated with, such therapies in their settings of practice. CONCLUSIONS: We conclude by proposing some key steps towards bridging the gaps in the management of HA with inhibitors in resource‐limited countries, including: (1) the collection of quality data that can inform healthcare reforms and policies; (2) improving disease knowledge among healthcare practitioners and patients with the aim of standardising disease management across centres and (3) working towards promoting equal access to HA care and therapies for everyone. John Wiley and Sons Inc. 2022-07-22 2022-11 /pmc/articles/PMC9796719/ /pubmed/35868021 http://dx.doi.org/10.1111/hae.14638 Text en © 2022 The Authors. Haemophilia published by John Wiley & Sons Ltd. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made. |
spellingShingle | Reviews Peyvandi, Flora Kavakli, Kaan El‐Beshlawy, Amal Rangarajan, Savita Management of haemophilia A with inhibitors: A regional cross‐talk |
title | Management of haemophilia A with inhibitors: A regional cross‐talk |
title_full | Management of haemophilia A with inhibitors: A regional cross‐talk |
title_fullStr | Management of haemophilia A with inhibitors: A regional cross‐talk |
title_full_unstemmed | Management of haemophilia A with inhibitors: A regional cross‐talk |
title_short | Management of haemophilia A with inhibitors: A regional cross‐talk |
title_sort | management of haemophilia a with inhibitors: a regional cross‐talk |
topic | Reviews |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9796719/ https://www.ncbi.nlm.nih.gov/pubmed/35868021 http://dx.doi.org/10.1111/hae.14638 |
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