Hypercoagulation Disorders
Hypercoagulation Disorders
Definition
Hypercoagulation disorders (or hypercoagulable states or disorders) have the opposite effect of the more common coagulation disorders. In hypercoagulation, there is an increased tendency for clotting of the blood, which may put a patient at risk for obstruction of veins and arteries (phlebitis or pulmonary embolism).
Description
In normal hemostasis, or the stoppage of bleeding, clots form at the site of the blood vessel's injury. The difference between that sort of clotting and the clotting present in hypercoagulation is that these clots develop in circulating blood.
This disorder can cause clots throughout the body's blood vessels, sometimes creating a condition known as thrombosis. Thrombosis can lead to infarction, or death of tissue, as a result of blocked blood supply to the tissue. However, hypercoagulability does not always lead to thrombosis. In pregnancy, and other hypercoagulable states, the incidence of thrombosis is higher than that of the general population, but is still under 10%. However, in association with certain genetic disorders, hypercoagulation disorders may be more likely to lead to thrombosis. Hypercoagulation disorders may also be known as hyperhomocystinemia, antithrombin III deficiency, factor V leyden, and protein C or protein S deficiency.
Causes and symptoms
Hypercoagulation disorders may be acquired or hereditary. Some of the genetic disorders that lead to hypercoagulation are abnormal clotting factor V, variations in fibrinogen, and deficiencies in proteins C and S. Other body system diseases may also lead to these disorders, including diabetes, sickle cell anemia, congenital heart disease, lupus, thalassemia, polycythemia rubra vera, and others. Antithrombin III deficiency is a hereditary hypercoagulation disorder that affects both sexes. Symptoms include obstruction of a blood vessel by a clot (thromboembolic disease), vein inflammation (phlebitis), and ulcers of the lower parts of the legs. The role of proteins C and S is a complex one. In order for coagulation to occur, platelets (small, round fragments in the blood) help contract blood vessels to lessen blood loss and also to help plug damaged blood vessels. However, the conversion of platelets into actual clots is a complicated web involving proteins that are identified clotting factors. The factors are carried in the plasma, or liquid portion of the blood. Proteins C and S are two of the clotting factors that are present in the plasma to help regulate or activate parts of the clotting process. Protein C is considered an anticoagulant. Mutation defects in the proteins may decrease their concentrations in the blood, and may or may not affect their resulting anticoagulant activity. Factor V is an unstable clotting factor also present in plasma. Abnormal factor V resists the changes that normally occur through the influence of protein C, which can also lead to hypercoagulability. Prothrombin, a glycoprotein that converts to thrombin in the early stage of the clotting process, is affected by the presence of these proteins, as well as other clotting factors.
Diagnosis
The diagnosis of hypercoagulation disorders is completed with a combination of physical examination, medical history, and blood tests. An accurate medical history is important to determine possible symptoms and causes of hypercoagulation disorders. There are a number of blood tests that can determine the presence or absence of proteins, clotting factors, and platelet counts in the blood. Among the tests used to detect hypercoagulation is the Antithrombin III assay. Protein C and Protein S concentrations can be diagnosed with immunoassay or plasma antigen level tests.
Treatment
Coumadin and heparin anticoagulants may be administered to reduce the clotting effects and maintain fluidity in the blood. Heparin is an anticoagulant that prevents thrombus formation and is used primarily for liver and lung clots.
Prognosis
The prognosis for patients with hypercoagulation disorders varies depending on the severity of the clotting and thrombosis. If undetected and untreated, thrombosis could lead to recurrent thrombosis and pulmonary embolism, a potentially fatal problem.
Prevention
Hereditary hypercoagulation disorders may not be prevented. Genetic and blood testing may help determine a person's tendency to develop these disorders.
Resources
ORGANIZATIONS
National Heart, Lung and Blood Institute. PO Box 30105, Bethesda, MD 20824-0105. (301) 251-1222. 〈http://www.nhlbi.nih.gov〉.
National Hemophilia Foundation. 116 West 32nd St., 11th Floor, New York, NY 10001. 800-424-2634. 〈http://www.hemophilia.org/home.htm〉.
KEY TERMS
Antithrombin— Any substance that counters the effect of thrombin, an enzyme that converts fibrinogen into fibrin, leading to blood coagulation.
Congenital— Refers to a condition or disorder present at birth.
Hemostasis— The arrest of bleeding.
Heparin— An anticoagulant, or blood clot "dissolver."
Polycythemia— A condition characterized by an overabundance of red blood cells.
Thalassemia— One of a group of inherited blood disorders characterized by a defect in the metabolism of hemoglobin, or the portion of the red blood cells that transports oxygen throughout the blood stream.
Thrombosis— Formation of a clot in the blood that either blocks, or partially blocks, a blood vessel. The thrombus may lead to infarction, or death of tissue due to a blocked blood supply.
Hypercoagulation Disorders
Hypercoagulation disorders
Description
Hypercoagulation disorders (or hypercoagulable states or disorders) cause an increased tendency for clotting of the blood. In normal hemostasis (the stoppage of bleeding) clots form at the site of the blood vessel's injury. However, in hypercoagulation disorders the clots can develop in circulating blood. This may put a patient at risk for obstruction of veins and arteries (phlebitis, thrombosis, or thrombophlebitis). The hypercoagulable state and thrombophlebitis is common cases of cancer involving solid tumors such as pancreatic, breast, ovarian, and prostate cancer .
Hypercoagulation disorders can cause clots throughout the body's blood vessels, a condition known as thromboembolic disease. Thromboembolic disease can lead to infarction (death of tissue as a result of blocked blood supply to the tissue). Other serious results of hypercoagulation make this a dangerous condition. Clotting (thrombosis) in the veins and arteries leading to the lungs can prevent blood flow, causing sudden and severe loss of breath and chest pain. These clots, called pulmonary embolisms, are potentially fatal. Clots in the blood vessels of the brain can result in a stroke, and clots in the heart's blood vessels can result in a heart attack.
Symptoms of hypercoagulation disorders include swelling or discoloration of the limbs, pain or tenderness of the skin, visible obstructions in the surface veins, and ulcers of the lower parts of the legs.
The diagnosis of hypercoagulation disorders is completed with a combination of physical examination, imaging studies , and blood tests. The presence of deep clots can be determined using Doppler ultrasound examination—special x-ray techniques called venography or arteriography (in which a solution is injected into the blood vessel to aid in imaging), or a specific type of blood pressure test called plethysmography. There are a number of blood tests that can determine the presence or absence of proteins, clotting factors, and platelet counts in the blood. Among the tests used to detect hypercoagulation is the Antithrombin III assay. Protein C and Protein S concentrations can be diagnosed with immunoassay or plasma antigen level tests.
Causes
Hypercoagulation disorders are associated with cancer of the pancreas. About half of patients with pancreatic cancer experience incidence of thrombosis. Approximately 10% of patients with pancreatic cancer develop a specific type of hypercoagulation disorder known as migratory thrombophlebitis, or Trousseau's syndrome. In Trousseau's syndrome the blood vessels become inflamed and clots in the blood vessels spontaneously appear and disappear. Other types of cancer may also result in hyper-coagulation disorders.
In order for blood coagulation to occur, platelets (small, round fragments in the blood) help contract blood vessels to lessen blood loss and also to help plug damaged blood vessels. The conversion of platelets into actual clots is a complicated process involving proteins that are identified clotting factors. The factors are carried in the plasma, or liquid portion, of the blood. Proteins C and S are two of the clotting factors that are present in the plasma to help regulate or activate parts of the clotting process. It is believed that pancreatic tumors produce chemicals that promote clotting, or coagulation, of the blood (procoagulants), or that they activate platelet function. It is also possible that tumors interfere with the functions of proteins C and S.
Treatments
The treatment for patients with hypercoagulation disorders varies depending upon the severity of the clotting and the other conditions it may have caused. Medications may include blood thinners (anticoagulants) such as heparin and warfarin , which prevent the formation of new blood clots; antiplatelet drugs such as aspirin; or thrombolytic drugs to dissolve existing clots. Pain medications and nonsteroidal anti-inflammatory medications may be given to reduce pain and swelling. Antibiotics will be prescribed if infection has occurred.
Resources
BOOKS
Deloughery, Thomas G. Hemostasis and Thrombosis. Georgetown, TX: Landes Bioscience, 1999.
Goodnight, Scott H., and William E. Goodnight. Disorders of Hemostasis and Thrombosis, 2nd ed. New York: McGraw-Hill, 2000.
ORGANIZATIONS
National Heart, Lung and Blood Institute. Building 31, Room4A21, Bethesda, MD 20892. (301) 496-4236. <http://www.nhlbi.nih.gov>
National Hemophilia Foundation. 116 West 32nd St., 11th Floor, New York, NY 10001. 800-42-HANDI. <http://www.hemophilia.org>
OTHER
American Academy of Family Physicians. Hypercoagulation: Excessive Blood Clotting. <http://familydoctor.org/handouts/244.html> (15 April 2001) 5 July 2001
Paul A. Johnson, Ed.M.
KEY TERMS
Antithrombin
—Any substance that counters the effect of thrombin. Thrombin is an enzyme that converts fibrinogen into fibrin, leading to blood coagulation.
Congenital
—A condition or disorder present at birth.
Hemostasis
—The arrest of bleeding.
Heparin
—An anticoagulant, or blood clot dissolver.
Phlebitis
—Inflammation of a vein.
Polycythemia
—A condition characterized by an overabundance of red blood cells.
Thrombophlebitis
—Inflammation of the vein with the formation of a thrombosis (blood clot).
Thrombosis
—Formation of a clot in the blood that either blocks, or partially blocks, a blood vessel. The thrombus may lead to infarction (death of tissue due to a blocked blood supply).