Hemophilia

Hemophilia is the name of several hereditary genetic illnesses that impair the body's ability to control bleeding, an impairment known technically as bleeding diathesis. Genetic deficiencies and a rare autoimmune disorder may cause lowered plasma clotting factor activity so as to compromise blood-clotting; when a blood vessel is injured, a scab does not form and the vessel continues to bleed for an excessive period of time. The bleeding can be external, if the skin is broken by a scrape, cut or abrasion, or it can be internal, into muscles, joints or hollow organs. The result may be visible or subtle.

Though there is no cure for hemophilia, it can be controlled with regular injections of the deficient clotting factor, i.e. factor VIII in hemophilia A or factor IX in hemophilia B. Some hemophiliacs develop antibodies against the replacement factors given to them, so the amount of the factor has to be increased or non-human replacement products must be given, such as porcine factor VIII.

If a patient becomes refractory to replacement coagulation factor as a result of circulating inhibitors, this may be overcome with recombinant human factor VII, which is registered for this indication in many countries.

In western countries, common standards of care fall into one of two categories: prophylaxis or on-demand. Prophylaxis involves the infusion of clotting factor on a regular schedule in order to keep clotting levels sufficiently high to prevent spontaneous bleeding episodes. On-demand treatment involves treating bleeding episodes once they arise.

...More at Wikipedia 

Related Links:

• National Hemophilia Foundation
• US National Library of Medicine
• Mayo Clinic

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