Uncommon inherited clotting disorders


  • Hemophilia is the most common hereditary clotting disorder (see also Overview of Blood Clotting Disorders). Hemophilia involves an inherited deficiency of certain clotting factors (proteins that help the blood clot, stopping bleeding). In hemophilia A, clotting factor VIII is deficient and in hemophilia B, factor IX is deficient. However, rarely people have an inherited deficiency of another clotting factor. Examples include a deficiency of factor II, V, VII, X, XI or XIII. Most of these disorders are inherited in an autosomal recessive fashion. In other words, the person needs to receive two copies of the abnormal gene, one from each parent.
    People with deficiencies of these clotting factors bruise easily and may have bleeding that is hard to stop (see also Bruising and Bleeding). Bleeding that is difficult to control after injury or surgery in a person who does not have hemophilia may indicate that a person has an uncommon clotting disorder. Doctors measure the levels of clotting factors in a blood sample in people who experience bruising or bleeding that is difficult to control.
    Treatment depends on the specific factor that is deficient but usually includes replacing the deficient clotting factor.


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