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A case series of acquired haemophilia in a Malaysian hospital: unpredictably rare medical emergency

Acquired haemophilia (AH) is a rare bleeding disorder characterized by the presence of acquired inhibitors against Factor VIII causing disruption of coagulation cascade. It has no known genetic inheritance, and diagnosis remains a challenge. The peculiar presentations are later age of onset as acute...

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Autores principales: Lee, Chiou Perng, Khalid, Bahariah Bt
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4626628/
https://www.ncbi.nlm.nih.gov/pubmed/26568837
http://dx.doi.org/10.1093/omcr/omv055
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author Lee, Chiou Perng
Khalid, Bahariah Bt
author_facet Lee, Chiou Perng
Khalid, Bahariah Bt
author_sort Lee, Chiou Perng
collection PubMed
description Acquired haemophilia (AH) is a rare bleeding disorder characterized by the presence of acquired inhibitors against Factor VIII causing disruption of coagulation cascade. It has no known genetic inheritance, and diagnosis remains a challenge. The peculiar presentations are later age of onset as acute pain in weight-bearing joints and spontaneous muscle haematoma with isolated prolonged activated partial thrombin time (APTT). Prevalence is 1 per million per year affecting both genders equally where blood product transfusion is seen in almost 87% of cases. The direct cause of AH is still unknown, and autoimmune dysregulation has been postulated, which predisposes to the development of the factor inhibitors. Being extremely rare, we are reporting two consecutive patients diagnosed by unusual bleeding episodes with isolated prolonged APTT due to Factor VIII inhibitors. AH deserves a special mention as high index of suspicion is required. More studies are required to provide better guidance in diagnosis and management of this condition.
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spelling pubmed-46266282015-11-13 A case series of acquired haemophilia in a Malaysian hospital: unpredictably rare medical emergency Lee, Chiou Perng Khalid, Bahariah Bt Oxf Med Case Reports Case Reports Acquired haemophilia (AH) is a rare bleeding disorder characterized by the presence of acquired inhibitors against Factor VIII causing disruption of coagulation cascade. It has no known genetic inheritance, and diagnosis remains a challenge. The peculiar presentations are later age of onset as acute pain in weight-bearing joints and spontaneous muscle haematoma with isolated prolonged activated partial thrombin time (APTT). Prevalence is 1 per million per year affecting both genders equally where blood product transfusion is seen in almost 87% of cases. The direct cause of AH is still unknown, and autoimmune dysregulation has been postulated, which predisposes to the development of the factor inhibitors. Being extremely rare, we are reporting two consecutive patients diagnosed by unusual bleeding episodes with isolated prolonged APTT due to Factor VIII inhibitors. AH deserves a special mention as high index of suspicion is required. More studies are required to provide better guidance in diagnosis and management of this condition. Oxford University Press 2015-10-06 /pmc/articles/PMC4626628/ /pubmed/26568837 http://dx.doi.org/10.1093/omcr/omv055 Text en © The Author 2015. Published by Oxford University Press. http://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/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Case Reports
Lee, Chiou Perng
Khalid, Bahariah Bt
A case series of acquired haemophilia in a Malaysian hospital: unpredictably rare medical emergency
title A case series of acquired haemophilia in a Malaysian hospital: unpredictably rare medical emergency
title_full A case series of acquired haemophilia in a Malaysian hospital: unpredictably rare medical emergency
title_fullStr A case series of acquired haemophilia in a Malaysian hospital: unpredictably rare medical emergency
title_full_unstemmed A case series of acquired haemophilia in a Malaysian hospital: unpredictably rare medical emergency
title_short A case series of acquired haemophilia in a Malaysian hospital: unpredictably rare medical emergency
title_sort case series of acquired haemophilia in a malaysian hospital: unpredictably rare medical emergency
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4626628/
https://www.ncbi.nlm.nih.gov/pubmed/26568837
http://dx.doi.org/10.1093/omcr/omv055
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