Hemophilia Health Channel

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Blood clots

Hemophilia A

Definition

Hemophilia A is a hereditary bleeding disorder caused by a lack of blood clotting factor VIII.

Causes, incidence, and risk factors

Hemophilia A results from a deficiency (lack) of clotting factor VIII.

The disorder is caused by an inherited X-linked recessive trait, with the defective gene located on the X chromosome. Females have two copies of the X chromosome, so if the factor VIII gene on one chromosome doesn't work, the gene on the other chromosome can do the job of making enough factor VIII. Males, however, have only one X chromosome, so if the factor VIII gene on that chromosome is broken, they will have hemophilia A. Thus, most people with hemophilia A are male.

If a woman has a defective factor VIII gene, she is considered a carrier. This means the defective gene can be passed down to her children. In a woman who carries the defective gene, any of her male children will have a 50% chance of having hemophilia A, while any of her female children will have a 50% chance of being a carrier. All female children of men with hemophilia carry the defective gene. Genetic testing is available for concerned parents.

The severity of symptoms can vary. Severe forms become apparent early on. Bleeding is the main symptom of the disease and sometimes, though not always, occurs if an infant is circumcised. Additional bleeding problems are seen when the infant starts crawling and walking.

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Bleeding disorders (Doctor-Reviewed information)
Bleeding disorders are a group of conditions in which there is a problem with the body''s blood clotting process. These disorders can lead to heavy and prolonged bleeding after an injury. Reviewer: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.Date: 03/02/2009

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