ACQUIRED HAEMOPHILIA A

Authors

  • Ewe Jin Koh Department of Internal Medicine, Hospital Taiping, Perak-Malaysia https://orcid.org/0000-0003-4580-0071
  • Jenq Tzong Tan Department of Internal Medicine, Hospital Taiping, Perak-Malaysia
  • Nur Nadia Amalina Manan Department of Internal Medicine, Hospital Taiping, Perak-Malaysia
  • Khai Yng Hor Department of Internal Medicine, Hospital Taiping, Perak-Malaysia
  • Zi Yu Teh Department of Internal Medicine, Hospital Taiping, Perak-Malaysia
  • Ming Lee Chin Department of Internal Medicine, Hospital Taiping, Perak-Malaysia

DOI:

https://doi.org/10.55519/JAMC-04-12289

Keywords:

Acquired Haemophilia, inhibitor, bruising, bleeding, coagulation, mixing test, bethesda unit

Abstract

Acquired haemophilia A (AHA) is a rare disease believed to be caused by spontaneous inhibition of clotting Factor VIII by autoantibodies. This is in contrast to the more common congenital haemophilias which are largely due to an absolute deficiency in coagulation factors. It has a prevalence of approximately one per million per year. However, this figure may be underestimated because there are many undocumented cases due to a lack of recognition. Patients who develop this disease may present with catastrophic bleeding despite having no previous bleeding history. In this study, we report a case of acquired Haemophilia A presenting with spontaneous unprovoked bruising and discuss the approach to diagnosis and how to alert the clinician to suspect this potentially rare but devastating disease.

Author Biographies

Ewe Jin Koh, Department of Internal Medicine, Hospital Taiping, Perak-Malaysia

 

Jenq Tzong Tan, Department of Internal Medicine, Hospital Taiping, Perak-Malaysia

 

Nur Nadia Amalina Manan, Department of Internal Medicine, Hospital Taiping, Perak-Malaysia

 

Khai Yng Hor, Department of Internal Medicine, Hospital Taiping, Perak-Malaysia

 

Zi Yu Teh, Department of Internal Medicine, Hospital Taiping, Perak-Malaysia

Medical Officer

Ming Lee Chin, Department of Internal Medicine, Hospital Taiping, Perak-Malaysia

 

References

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Tiede A, Collins P, Knoebl P, Teitel J, Kessler C, Shima M, et al. International recommendations on the diagnosis and treatment of acquired hemophilia A. Haematologica 2020;105(7):1791–801.

Knoebl P, Marco P, Baudo F, Collins P, Huth-Kühne A, Nemes L, et al. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2): Demographics of acquired hemophilia A. J Thromb Haemost [Internet]. 2012;10(4):622–31. Available from: http://dx.doi.org/10.1111/j.1538-7836.2012.04654.x

Pishko AM, Doshi BS. Acquired hemophilia A: Current guidance and experience from clinical practice. J Blood Med [Internet]. 2022;13:255–65. Available from: http://dx.doi.org/10.2147/JBM.S284804

Tiede A, Klamroth R, Scharf RE, Trappe RU, Holstein K, Huth-Kühne A, et al. Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from the GTH-AH 01/2010 study. Blood [Internet]. 2015;125(7):1091–7. Available from: http://dx.doi.org/10.1182/blood-2014-07-587089

Haider MZ, Anwer F. Acquired Hemophilia. StatPearls Publishing; 2022.

Lossing TS, Kasper CK, Feinstein DI. Detection of factor VIII inhibitors with the partial thromboplastin time. Blood [Internet]. 1977;49(5):793–7. Available from: http://dx.doi.org/10.1182/blood.v49.5.793.793

Ewenstein BM. Nonhemophilic Inhibitors of Coagulation. In: Consultative Hemostasis and Thrombosis. Elsevier; 2007. p. 81–95.

Tiede A, Collins P, Knoebl P, Teitel J, Kessler C, Shima M, et al. International recommendations on the diagnosis and treatment of acquired hemophilia A. Haematologica [Internet]. 2020;105(7):1791–801. Available from: http://dx.doi.org/10.3324/haematol.2019.230771

Additional Files

Published

2023-12-23