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  Disease von Willebrands

Disease von Willebrands is a blood coagulation disease that causes bleeding disorders and can be serious or undiagnosed. The von Willebrand factor is a protein combination called a multimeric protein or a blood glycoprotein. A glycoprotein is a combination of a carbohydrate and a protein. The primary purpose of the von Willebrand factor is to bind cells together, particularly at the site of a cut or injury resulting in bleeding.

Disease von Willebrands
Disease von Willebrands


Disease Von Willebrands is the most common heredity disease of the blood that affects coagulation. Coagulation is the natural process of blood that creates clots. Disease von Willebrands has been found in both humans and dogs.

Disease von Willebrands was first described in 1926 by pediatrician Erik Adolf von Willebrand of Finland.

Symptoms:

Disease von Willebrands has degrees of symptoms. It manifests as a bleeding disorder. In women, it can mean extremely heavy bleeding during menstruation and childbirth. It can also, though rarely be present as bleeding in the joints and other internal bleeding. Unusual bruising and nosebleeds are also symptoms of von Willebrands disease.

Diagnosis:

Disease von Willebrands is caused by a quantitative lack of the von Willebrand Factor or a poor quality (abnormal) von Willebrand factor in the blood plasma. To diagnose a patient suspected of having Disease von Willebrands, the blood plasma must be examined. The quality and quantity of the von Willebrand factor is assessed. The amount of vWF(von Willebrand Factor) can be measured using a vWF antigen assay. The quality or functioning of the vWF is measured with three tests that specifically look at the factor. Those three tests are glycoprotein binding assay, a ristocetin cofactor activity assay and a collagen binding assay. When checking the quality of the vWF, Factor VIII can be examined. Factor VIII binds with vWF – the vWF is a preservative or protectorate of Factor VIII. A lower Factor VIII level indicates a possible lower vWF level.

A full blood count – including platelet counts, an activated partial thromboplastin time, a prothrombin time and a fibrinogen level are also blood evaluations performed when diagnosing a patient with coagulation disorders. Depending on the results, further coagulation studies may be required for the patient.

Treatment:

There are three classifications of hereditary Disease von Willebrands. A fourth type of Disease von Willebrands is called Acquired Disease von Willebrands. These classifications have been defined by the International Society on Thrombosis and Haemostasis's (ISTH) and are based on the quality and quantity of vWF.

The three classifications of hereditary Disease von Willebrands are types 1, 2 and 3. Type 1 vWD is the most common type in vWD patients, occurring in almost eighty percent of diagnosed patients. This form of vWD is a quantitative issue of the vWF, in which vWF level is ten to forty percent less than typical blood levels. Type 2 vWD has four subtypes – based on qualitative challenges of the vWF – they are known as 2A, 2B, 2M and 2N. Type 3 is the most severe form of Disease von Willebrand. This type has a homozygous defective gene and presents as Type 3 is the most severe form of vWD (homozygous for the defective gene) and may have relentless bleeding of mucus membranes. In patient with type 3, there is no detectable vWF antigen and low factor VIII – if presents a a case of mild hemophilia.

Acquired vWD occurs in patients where the vWF is not subdued, but is rapidly cleared from circulation of the body. A form of this occurs in patients with aortic valve stenosis (abnormal constriction). It leads to Heyde’s syndrome – a gastrointestinal bleeding.

Typically vWD patients don not need regular treatment. There is always a dramatic risk of bleeding. The common treatment is a prophylactic infusion of human derived medium purity Factor VIII concentrate before surgery. Cases that are mild have had trial treatments of desmopressin, a drug that raises a patient’s plasma level.

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