von Willebrand’s disease - symptoms and treatment
What is von Willebrand’s disease and Definition
A hereditary, genetic bleeding disorder resulting from a deficiency or molecular abnormality of clotting factor VIII. Unlike HEMOPHILIA A, which also results from clotting factor VIII deficiency, von Willebrand’s disease affects both men and women equally. Its inheritance pattern is autosomal dominant, meaning a child can acquire the condition when only one parent has the defective GENE. Von Willebrand’s disease is the most common bleeding disorder in the United States, affecting about 1 percent of the American population. The condition is mild in most people, though severe trauma or major surgery may cause life-threatening hemorrhage especially in people who do not know they have the disorder.
Symptoms of von Willebrand’s disease and Diagnostic Path
The most common symptom of von Willebrand’s disease is somewhat prolonged bleeding with cuts, wounds, dental procedures, and surgeries. Easy bruising, frequent nosebleeds (EPISTAXIS), and bleeding gums are also common symptoms. Some people may periodically develop PETECHIAE, pinpoint hemorrhages beneath the surface of the SKIN that have the appearance of a RASH. Women who have von Willebrand’s disease may have unusually heavy menstrual bleeding. The diagnostic path includes blood tests to measure clotting times, PLATELET AGGREGATION, and the level of von Willebrand factor multimers in the blood. The results of these diagnostic blood tests are usually conclusive for the diagnosis.
Von Willebrand’s disease Treatment Options and Outlook
No treatment is necessary for people who have mild symptoms, though anyone diagnosed with von Willebrand’s disease should carry or wear identification that alerts emergency medical personnel to the condition. Treatment before scheduled surgeries or for bleeding due to trauma is administration of PLASMA cryoprecipitate, which contains concentrated CLOTTING FACTORS, or purified factor VIII concentrate.
People who have von Willebrand’s disease should not take NONSTEROIDAL ANTIINFLAMMATORY DRUGS (NSAIDS), including aspirin, as these medications further decrease PLATELET AGGREGATION and increase bleeding.
Risk Factors and Preventive Measures
As von Willebrand’s disease is hereditary, family history is the only known risk factor. People who have von Willebrand’s disease may choose GENETIC COUNSELING before deciding to conceive children. Most people who have von Willebrand’s disease experience little interference with QUALITY OF LIFE. The condition generally remains stable throughout life. Other health conditions that affect bleeding may result in a compound effect to produce more intense symptoms than either condition alone would otherwise manifest.
See also COAGULATION; GENETIC DISORDERS.
