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Hemophilia is the name of any of several hereditary genetic illnesses that impair the body's ability to control bleeding and blood loss. Genetic deficiencies (or, very rarely, an autoimmune disorder) cause lowered plasma clotting factor activity so as to hinder blood-clotting.
In normal individuals, when a blood vessel is injured a scab will form and bleeding will stop. With Hemophilia, when a blood vessel is injured, a scab will not form and the vessel can continue to bleed excessively for a very long period of time, often until all blood is lost. 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.
There are three main types of hemophilia
Hemophilia A: Hemophilia leads to a severely increased risk of bleeding from common injuries. The most common sites of bleeding are:
- digestive tract
The muscle and joint haemorrhages are quite typical of hemophilia, while digestive tract and cerebral haemorrhages are also typical of other coagulation disorders.
Hemophilia B: Deficiency of the Factor IX gene located on the X chromosome, leads to an increased propensity for haemorrhage. Bleeding may be in response to mild trauma or even spontaneously, such as in joints (haemarthrosis) or muscles.
Hemophilia C: is a mild form of hemophilia that mainly occurs in Jews of Ashkenazi descent. It is due to deficiency of coagulation factor XI. Treatment is usually not necessary, except in relation to operations.
Posted by Staff at July 6, 2005 5:57 AMblog comments powered by Disqus