Hemophilia
Hemophilia is a topic covered in the 5-Minute Emergency Consult.
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Basics
Description
Description
- Caused by deficiency of functional factor VIII or factor IX
- Lack of functional factor causes partial inactivation of coagulation cascade and impaired hemostasis.
- 2 types:
- Hemophilia A: Factor VIII deficiency
- Hemophilia B (Christmas disease): Factor IX deficiency
- Symptoms dependent on factor activity:
- 5–30% factor activity (mild hemophilia):
- Bleeding with major trauma or surgery
- 1–5% factor activity (moderate hemophilia):
- Bleeding secondary to trauma/surgery
- Occasional spontaneous hemarthroses (<1 time per month)
- <1% factor activity (severe):
- Spontaneous bleeding from infancy
- May bleed as often as 1–2 times per week, often requiring factor replacement
- 5–30% factor activity (mild hemophilia):
- Complications:
- Death from hemorrhage
- Recurrent joint bleeding leads to joint destruction and loss of function
- Transfusion-transmitted infections (risk reduced with purification of concentrates)
- Development of inhibitors (IgG antibodies to factors), which prevent hemostasis
Etiology
GeneticsEtiology
- X-linked recessive, though 1/3 have a spontaneous mutation
- Rare disease:
- Hemophilia A: 1 in 5–7,000 males
- Hemophilia B: 1 in 30,000 males
- Prevalence of inhibitors: 20% of severe hemophilia A and <5% of severe hemophilia B
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Citation
Rosen, Peter, et al., editors. "Hemophilia." 5-Minute Emergency Consult, 5th ed., Lippincott Williams & Wilkins, 2016. Emergency Central, emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307298/all/Hemophilia.
Hemophilia. In: Rosen P, Shayne P, Barkin AZ, et al, eds. 5-Minute Emergency Consult. 5th ed. Lippincott Williams & Wilkins; 2016. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307298/all/Hemophilia. Accessed December 9, 2019.
Hemophilia. (2016). In Rosen, P., Shayne, P., Barkin, A. Z., Wolfe, R. E., Hayden, S. R., Barkin, R. M., & Schaider, J. J. (Eds.), 5-Minute Emergency Consult. Available from https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307298/all/Hemophilia
Hemophilia [Internet]. In: Rosen P, Shayne P, Barkin AZ, Wolfe RE, Hayden SR, Barkin RM, Schaider JJ, editors. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2016. [cited 2019 December 09]. Available from: https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307298/all/Hemophilia.
* Article titles in AMA citation format should be in sentence-case
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