haemophilia

Any of several inherited diseases in which normal blood clotting is impaired. The sufferer experiences prolonged bleeding from the slightest wound, as well as painful internal bleeding without apparent cause.

Haemophilias are nearly always sex-linked, transmitted through the female line only to male infants; they have afflicted a number of European royal households. Males affected by the most common form are unable to synthesize Factor VIII, a protein involved in the clotting of blood. Treatment is primarily with Factor VIII (now mass-produced by recombinant techniques), but the haemophiliac remains at risk from the slightest incident of bleeding. The disease is a painful one that causes deformities of joints.

An artificial form of Factor VIII went on sale in 1993 in Germany and Sweden, and in the UK in 1994.