Sometimes a malfunction of the body's immune system causes it to make antibodies that attack the person's own tissues (autoantibodies) instead of attacking foreign or dangerous substances. Such disorders are called autoimmune disorders Autoimmune Disorders An autoimmune disorder is a malfunction of the body's immune system that causes the body to attack its own tissues. What triggers an autoimmune disorder is not known. Symptoms vary depending... read more .
Circulating anticoagulants are usually autoantibodies that develop spontaneously (on their own and without a known cause) and decrease the activity of a specific clotting factor (a protein that helps the blood clot) and, therefore, cause excessive bleeding Bruising and Bleeding Bruising or bleeding after an injury is normal (see also How Blood Clots). However, some people have disorders that cause them to bruise or bleed too easily. Sometimes people bleed without any... read more (see also Overview of Blood Clotting Disorders Overview of Blood Clotting Disorders Blood clots help stop bleeding. Blood clotting (coagulation) disorders are dysfunctions in the body's ability to control the formation of blood clots. These dysfunctions may result in Too little... read more ). For example, the immune system may produce an autoantibody against clotting factor VIII or factor V. These antibodies are called circulating anticoagulants because they travel (circulate) in the bloodstream.
Circulating anticoagulants usually cause excessive bleeding. However, some people with certain types of circulating anticoagulants develop blood clots within an artery or vein instead of excessive bleeding. Such blood clots may cut off blood flow, causing redness and swelling as well as damage to the tissue supplied by the blood vessel.
Circulating anticoagulants are suspected in patients with excessive bleeding Bruising and Bleeding Bruising or bleeding after an injury is normal (see also How Blood Clots). However, some people have disorders that cause them to bruise or bleed too easily. Sometimes people bleed without any... read more combined with abnormal coagulation test results.
Treatment may include medications to suppress autoantibody production in patients without hemophilia.
Factor VIII and factor IX inhibitors
Antibodies to factor VIII develop in about 30% of people with severe hemophilia A Hemophilia Hemophilia is a hereditary bleeding disorder caused by a deficiency in one of two blood clotting factors: factor VIII or factor IX. Several different gene abnormalities can cause the disorder... read more . The antibodies develop as a complication of repeated exposure to normal factor VIII molecules that are given to treat the hemophilia. Similarly, people with severe hemophilia B Hemophilia Hemophilia is a hereditary bleeding disorder caused by a deficiency in one of two blood clotting factors: factor VIII or factor IX. Several different gene abnormalities can cause the disorder... read more can develop antibodies to factor IX, although this occurs in only about 2 to 3% of people.
Factor VIII autoantibodies also arise occasionally in people without hemophilia. For example, factor VIII anticoagulants may develop in women who have just given birth or in people who have an autoimmune disorder, such as rheumatoid arthritis Rheumatoid Arthritis (RA) Rheumatoid arthritis is an inflammatory arthritis in which joints, usually including those of the hands and feet, are inflamed, resulting in swelling, pain, and often destruction of joints.... read more or systemic lupus erythematosus Systemic Lupus Erythematosus (SLE) Systemic lupus erythematosus is a chronic autoimmune inflammatory connective tissue disorder that can involve joints, kidneys, skin, mucous membranes, and blood vessel walls. Problems in the... read more . Sometimes, older people who have no apparent underlying disorder develop factor VIII anticoagulants.
People with a factor VIII anticoagulant can develop life-threatening bleeding.
Blood tests are done, including measurement of the amount of factor VIII or the amount of factor IX.
In postpartum women, the autoantibodies may disappear spontaneously. Otherwise, people without hemophilia may be given drugs such as cyclophosphamide, corticosteroids, or rituximab to suppress autoantibody production. People with antibodies to factor VIII who are bleeding may also be given activated factor VII.
Several medications are available or being tested for use to control bleeding in people with hemophilia and isoantibodies to factor VIII or factor IX.