Haemophilia is a genetic disorder of haemostasis, that is, the blood clotting process. It is due to the lack or deficiency of a blood protein called clotting factor. In the blood plasma there are several factors (13 in total), which are called coagulation factors I-XIII and are all necessary for the haemostatic process. When one of these coagulation factors is absent or in low levels, the blood does not clot quickly, resulting in the person bleeding for longer time than other people do.
Haemophilia occurs more frequently in males than females, as is genetically transferred from mother to son. Therefore, the person is born with the disorder and lives with it throughout his or her life. The chance of giving birth to a girl with haemophilia is very rare.
In haemophilia A there is lack or deficiency of clotting factor VIII (Factor 8). It is the most common type of haemophilia as it accounts for about 80% of haemophilia cases. . Haemophilia A occurs in 1 in 5,000 newborn boys or in 1 in 10,000 births.
Haemophilia B (also known as Christmas disease) is caused by the lack or deficiency of clotting factor IX (factor 9)..
Haemophilia is diagnosed by blood tests that measure platelet count, blood clotting time, prothrombin time (PT) and partial thromboplastin time (aPTT), followed by measurements of factor VIII or factor IX levels. The diagnosis of mild or severe haemophilia is usually made in infancy (0-2 years old), as the baby's parents observe bruises on their hands or feet, especially when they start crawling or walking. Haemophilia should not be confused with von Willebrand disease, a bleeding disorder due to the lack or defective function of a different type of clotting factor, calledvon Willebrand factor (VWF).
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1.WFH. What are bleeding disorders? 2016.
Available at: http://www.wfh.org/en/page.aspx?pid=1282 [Accessed 24 October 2019]