Hemophilia

Learning objectives

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%

Hemophilia	Mild	Moderate	Severe
% activity of factors Factor levels (IU/ml)	5-40 0.05-0.40	1-5 0.01-0.05	<1 <0.01

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:
- Pregnancy
- Autoimmune disorders
- Cancer
- Multiple sclerosis
- Drug reactions