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%

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

Causes

• Congenital:
  • Caused by an X-linked genetic mutation
  • Occurs in 1/5000 male births
• Acquired hemophilia:
  • Pregnancy
  • Autoimmune disorders
  • Cancer
  • Multiple sclerosis
  • Drug reactions

Complications

• Deep internal bleeding
• Bleeding into the throat or neck
• Damage to joints
• Infection: hepatitis C
• Adverse reaction to clotting factor treatment

Management

Specific treatment

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.

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