Definition
Coagulation disorders deal with disruption of the body's ability to control blood clotting. The most commonly known coagulation disorder is hemophilia, a condition in which patients bleed for long periods of time before clotting. There are other coagulation disorders with a variety of causes.
Description
Coagulation, or clotting, occurs as a complex process involving several components of the blood. Plasma, the fluid component of the blood, carries a number of proteins and coagulation factors that regulate bleeding. Platelets, small colorless fragments in the blood, initiate contraction of damaged blood vessels so that less blood is lost. They also help plug damaged blood vessels and work with plasma to accelerate blood clotting. A disorder affecting platelet production or one of the many steps in the entire process can disrupt clotting.
Coagulation disorders arise from different causes and produce different complications. Some common coagulation disorders are:
• Hemophilia, or hemophilia A (Factor VIII deficiency), an inherited coagulation disorder, affects about 20,000 Americans. This genetic disorder is carried by females but most often affects males.
• Christmas disease, also known as hemophilia B or Factor IX deficiency, is less common than hemophilia A with similar in symptoms.
• Disseminated intravascular coagulation disorder, also known as consumption coagulopathy, occurs as a result of other diseases and conditions. This disease accelerates clotting, which can actually cause hemorrhage.
• Thrombocytopenia is the most common cause of coagulation disorder. It is characterized by a lack of circulating platelets in the blood. This disease also includes idiopathic thrombocytopenia.
• Von Willebrand's disease is a hereditary disorder with prolonged bleeding time due to a clotting factor deficiency and impaired platelet function. It is the most common hereditary coagulation disorder.
• Hypoprothrombinemia is a congenital deficiency of clotting factors that can lead to hemorrhage.
• Other coagulation disorders include Factor XI deficiency, also known as hemophilia C, and Factor VII deficiency. Hemophilia C afflicts one in 100,000 people and is the second most common bleeding disorder among women. Factor VII is also called serum prothrombin conversion accelerator (SPCA) deficiency. One in 500,000 people may be afflicted with this disorder that is often diagnosed in newborns because of bleeding into the brain as a result of traumatic delivery.
Causes and symptoms
Some coagulation disorders present symptoms such as severe bruising. Others will show no apparent symptoms, but carry the threat of severe internal bleeding.