Hemophilia - NYSORA

Explore NYSORA knowledge base for free:


Learning objectives

  • Definition and types of hemophilia
  • Management of hemophilia

Definition and mechanisms

  • Inherited bleeding disorder in which the blood does not clot properly
  • Resulting in a longer bleeding time: ↑ PTT, normal INR 
  • Spontaneous bleeding as well as bleeding following injuries or surgery
  • Severity depends on the amount of factor in the blood 
  • Two types of hemophilia:
    • Hemophilia A (Classic Hemophilia): caused by a lack or decrease of clotting factor VIII
    • Hemophilia B (Christmas Disease): caused by a lack or decrease of clotting factor IX
    • Hemophilia is 4 times as common as hemophilia B
    • A normal value of coagulation factor is 0.5-1.5 IU/ml or 50-150%
% activity of factors
Factor levels (IU/ml)

Signs and symptoms

  • Unexplained and excessive bleeding from cuts or injuries, or after surgery or dental work
  • Many large or deep bruises
  • Unusual bleeding after vaccinations
  • Pain, swelling, or tightness in the joints
  • Blood in your urine or stool
  • Nosebleeds without a known cause
  • Unexplained irritability in infants


  • Congenital:
    • Caused by an X-linked genetic mutation
    • Occurs in 1/5000 male births
  • Acquired hemophilia:



Hemophilia, factor VIII, factor IX, fibrinogen, HIV, hepatitis B, hepatitis C

Specific treatment

Specific treatment of hemophilia, factor VIII, Factor IX, desmopressin, tranexamic acid

Suggested reading

Shah UJ, Madan Narayanan M, J Graham Smith JH. Anaesthetic considerations in patients with inherited disorders of coagulation, Continuing Education in Anaesthesia Critical Care & Pain, Volume 15, Issue 1, February 2015, Pages 26–31.

We would love to hear from you. If you should detect any errors, email us customerservice@nysora.com