A case series of acquired haemophilia in a Malaysian hospital: unpredictabty 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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Main Authors: Chiou, Perng Lee, Khalid, Bahariah
Format: Article
Language:English
Published: Oxford University Press 2015
Online Access:http://psasir.upm.edu.my/id/eprint/43462/
http://psasir.upm.edu.my/id/eprint/43462/1/abstract123.pdf
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author Chiou, Perng Lee
Khalid, Bahariah
author_facet Chiou, Perng Lee
Khalid, Bahariah
author_sort Chiou, Perng Lee
building UPM Institutional Repository
collection Online Access
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 upm-434622016-06-28T03:35:03Z http://psasir.upm.edu.my/id/eprint/43462/ A case series of acquired haemophilia in a Malaysian hospital: unpredictabty rare medical emergency Chiou, Perng Lee Khalid, Bahariah 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 Article PeerReviewed application/pdf en http://psasir.upm.edu.my/id/eprint/43462/1/abstract123.pdf Chiou, Perng Lee and Khalid, Bahariah (2015) A case series of acquired haemophilia in a Malaysian hospital: unpredictabty rare medical emergency. Oxford Medical Case Reports, 2015 (10). pp. 330-332. ISSN 2053-8855 10.1093/omcr/omv055
spellingShingle Chiou, Perng Lee
Khalid, Bahariah
A case series of acquired haemophilia in a Malaysian hospital: unpredictabty rare medical emergency
title A case series of acquired haemophilia in a Malaysian hospital: unpredictabty rare medical emergency
title_full A case series of acquired haemophilia in a Malaysian hospital: unpredictabty rare medical emergency
title_fullStr A case series of acquired haemophilia in a Malaysian hospital: unpredictabty rare medical emergency
title_full_unstemmed A case series of acquired haemophilia in a Malaysian hospital: unpredictabty rare medical emergency
title_short A case series of acquired haemophilia in a Malaysian hospital: unpredictabty rare medical emergency
title_sort case series of acquired haemophilia in a malaysian hospital: unpredictabty rare medical emergency
url http://psasir.upm.edu.my/id/eprint/43462/
http://psasir.upm.edu.my/id/eprint/43462/
http://psasir.upm.edu.my/id/eprint/43462/1/abstract123.pdf