Doctor Examining Litle Boy

Haemophilia Overview

Haemophila is an inherited, x-linked lifelong bleeding disorder which affects males almost exclusively. Most frequently haemorrhage involves joints or muscles. Bleeding patterns differ with age: infants usually bleed into soft tissues or from the mouth but as the boy grows, characteristic joint bleeding becomes more common.
l Haemophilia A (or classical haemophilia) is the most common form and is due to a deficiency of clotting factor VIII.
l Haemophilia B (or Christmas disease) is due to a deficiency of clotting factor IX.
l Severity -- Haemophilia is classified as severe, moderate or mild, which indicates the expected frequency of bleeding.
l Severe: factor VIII or IX < 2%
F VIII or IX replacement is usually needed several times per month for traumatic or apparent spontaneous bleeding.
l Moderate: factor VIII or IX 2-5%
Less frequent bleeding which usually follows trauma, surgery or dental work.
l Mild: factor VIII or IX 5-40%
Occasional bleeding, usually only after severe trauma or surgery.

FACTOR INHIBITORS
Develop in about 15% of boys with haemophilia A, but very rarely in haemophilia B. Inhibitors are antibodies which neutralise the infused coagulation factor. This is a serious complication because therapy of f VIII or f IX is rendered ineffective.

Treatment is simple: Replace the missing clotting factor.

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