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Hemophilia

Hemophilia is a topic covered in the 5-Minute Emergency Consult.

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Basics

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
  • 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

Genetics
  • 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 April 24, 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 April 24]. 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
TY - ELEC T1 - Hemophilia ID - 307298 ED - Rosen,Peter, ED - Shayne,Philip, ED - Barkin,Adam Z, ED - Wolfe,Richard E, ED - Hayden,Stephen R, ED - Barkin,Roger M, ED - Schaider,Jeffrey J, BT - 5-Minute Emergency Consult UR - https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307298/all/Hemophilia PB - Lippincott Williams & Wilkins ET - 5 DB - Emergency Central DP - Unbound Medicine ER -