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SR Vol.47(03) [March 2010] >

Title: Hostile Hemophilia
Authors: Chawla, P. Cheena
Issue Date: Mar-2010
Publisher: CSIR
Abstract: Adequate government support coupled with public awareness is crucial to tackle the myriad concerns about hemophilia.

BLOOD — the river of life — flows through a network of channels spread all over the human body called blood vessels, which bring to the doorstep of every cell the right amounts of food and oxygen. The sight of oozing blood, whether it is a mild bruise or a serious injury, is truly distressing, particularly if bleeding occurs non-stop. It could prove fatal in no time if the natural clotting power of the blood is flawed. Millions of victims of a genetic disorder called hemophilia bear the harassment of unstoppable bleeding even for a minor wound, as their blood lacks certain crucial proteins that normally work with special cells in blood — the platelets — till the wound is finally sealed to form a clot. A child born with hemophilia simply lacks clotting factors that help platelets stick together to plug the cuts at the site of injury to stop bleeding. This happens as the child inherits defective genes for these clotting factors from one or both parents. There are two main types of hemophilia: hemophilia A (classic hemophilia) where there are low levels of clotting factor VIII and hemophilia B (Christmas disease) where victims have abysmally low levels of clotting factor IX. About 9 out of 10 victims suffer from hemophilia A. Depending on the level of deficiency of these clotting factors, hemophilia is classified as mild, moderate or severe. About 7 out of 10 people who have hemophilia A have the severe form of the disorder. In some persons with hemophilia A, the Factor VIII replacement therapy is identified as a foreign substance by their defence machinery. When this happens, antibodies are made against Factor VIII, which inhibit the ability of the infused Factor VIII to work in the clotting process. This faulty process of raising an immune response against body’s own proteins is called autoimmunity. Clotting factor levels are also measured when a patient is suspected of having an acquired condition that is causing excessive bleeding, such as vitamin K deficiency, liver disease or cancer to name a few.

Page(s): 40-45
Source:SR Vol.47(03) [March 2010]

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