Von Willebrand's Disease (VWD)

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Disease Ontology 11 DOID:12531 ICD9CM 34 286.4 MeSH 43 D014842 NCIt 49 C68677 SNOMED-CT 68 11093006 ICD10 31 D68.0 MESH via Orphanet 44 D014842

ICD10 via Orphanet 32 D68.0 UMLS via Orphanet 72 C0042974 Orphanet 58 ORPHA903 SNOMED-CT via HPO 69 203556007 20696009 26517000 362970003 64779008 more ICD11 33 2112021600 UMLS 71 C0042974

MedlinePlus Genetics: ⁴² Von Willebrand disease is a bleeding disorder that slows the blood clotting process, causing prolonged bleeding after an injury. People with this condition often experience easy bruising, long-lasting nosebleeds, and excessive bleeding or oozing following an injury, surgery, or dental work. Mild forms of von Willebrand disease may become apparent only when abnormal bleeding occurs following surgery or a serious injury. Women with this condition typically have heavy or prolonged bleeding during menstruation (menorrhagia), and some may also experience reproductive tract bleeding during pregnancy and childbirth. In severe cases of von Willebrand disease, heavy bleeding occurs after minor trauma or even in the absence of injury (spontaneous bleeding). Symptoms of von Willebrand disease may change over time. Increased age, pregnancy, exercise, and stress may cause bleeding symptoms to become less frequent.Von Willebrand disease is divided into three types, with type 2 being further divided into four subtypes. Type 1 is the mildest and most common of the three types, accounting for 75 percent of affected individuals. Type 3 is the most severe and rarest form of the condition. The four subtypes of type 2 von Willebrand disease are intermediate in severity. Another form of the disorder, acquired von Willebrand syndrome, is not caused by inherited gene mutations. Acquired von Willebrand syndrome is typically seen along with other disorders, such as diseases that affect bone marrow or immune cell function. This rare form of the condition is characterized by abnormal bleeding into the skin and other soft tissues, usually beginning in adulthood.

MalaCards based summary: Von Willebrand's Disease, also known as von willebrand disease, is related to von willebrand disease, type 2 and von willebrand disease, type 3. An important gene associated with Von Willebrand's Disease is VWF (Von Willebrand Factor), and among its related pathways/superpathways are Disease and Response to elevated platelet cytosolic Ca2+. The drugs Arginine and Arginine Vasopressin have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, bone and skin, and related phenotypes are abnormal platelet function and abnormality of coagulation

PubMed Health : ⁶³ Von willebrand disease: Von Willebrand disease (VWD) is a bleeding disorder. It affects your blood's ability to clot. If your blood doesn't clot, you can have heavy, hard-to-stop bleeding after an injury. The bleeding can damage your internal organs. Rarely, the bleeding may even cause death. In VWD, you either have low levels of a certain protein in your blood or the protein doesn't work well. The protein is called von Willebrand factor, and it helps your blood clot. Normally, when one of your blood vessels is injured, you start to bleed. Small blood cell fragments called platelets (PLATE-lets) clump together to plug the hole in the blood vessel and stop the bleeding. Von Willebrand factor acts like glue to help the platelets stick together and form a blood clot. Von Willebrand factor also carries clotting factor VIII (8), another important protein that helps your blood clot. Factor VIII is the protein that's missing or doesn't work well in people who have hemophilia, another bleeding disorder. VWD is more common and usually milder than hemophilia. In fact, VWD is the most common inherited bleeding disorder. It occurs in about 1 out of every 100 to 1,000 people. VWD affects both males and females, while hemophilia mainly affects males.

CDC: ² Von Willebrand disease (VWD) is a blood disorder in which the blood does not clot properly. Blood contains many proteins that help the body stop bleeding. One of these proteins is called von Willebrand factor (VWF). People with VWD either have a low level of VWF in their blood or the VWF protein doesn't work the way it should. Normally, when a person is injured and starts to bleed, the VWF in the blood attaches to small blood cells called platelets. This helps the platelets stick together, like glue, to form a clot at the site of injury and stop the bleeding. When a person has VWD, because the VWF doesn't work the way it should, the clot might take longer to form or not form the way it should, and bleeding might take longer to stop. This can lead to heavy, hard-to-stop bleeding. Although rare, the bleeding can be severe enough to damage joints or internal organs, or even be life-threatening.

GARD: ¹⁹ Von Willebrand disease is a bleeding disorder that slows the blood clotting process. People with this disease often experience bruising, nosebleeds, and prolonged bleeding or oozing following an injury, affer surgery, or having a tooth pulled. Affected women may have heavy menstrual bleeding. In severe cases, heavy bleeding occurs after minor injury or even in the absence of injury. It is divided into three types. Type 1 is the mildest and most common, and type 3 is the most severe and rarest form. Increased age, pregnancy, exercise, and stress may cause von Willebrand factor levels in the blood to rise, which can make bleeding symptoms less frequent. This disease is caused by genetic changes in the VWF gene and can have different inheritance patterns.

Orphanet: ⁵⁸ A rare, inherited bleeding disorder characterized by defective platelet adhesion and secondary coagulation defect that manifests as abnormal bleeding of variable severity occurring either spontaneously or in association with an invasive procedure. Three main subtypes are defined based on the type of von Willebrand factor defect: partial (type 1) or total (type 3) deficiency, and qualitative/functional anomalies (type 2).

Disease Ontology: ¹¹ A blood coagulation disease that is a hereditary abnormality which slows the blood clotting process. It arises from a qualitative or quantitative deficiency of von Willebrand factor (vWF), a multimeric protein that is required for platelet adhesion.

Wikipedia: ⁷⁵ Von Willebrand disease (VWD) is the most common hereditary blood-clotting disorder in humans. An... more...

GeneReviews: NBK7014

#	Description	GenomeRNAi Source Accession	Score	Top Affiliating Genes
1	no effect	GR00402-S-1	10.19	ADAMTS13 CD63 F11 F2 F3 F5
2	no effect	GR00402-S-2	10.19	ADAMTS13 CD63 F2 F3 F5 F7

Treatment for von Willebrand disease (VWD) is based on the type of VWD you have and how severe it is. Most cases of VWD are mild, and you may need treatment only if you have surgery , tooth extraction, or an accident. Medicines are used to: Increase the amount of von Willebrand factor and factor VIII released into the bloodstream Replace von Willebrand factor Prevent the breakdown of blood clots Control heavy menstrual bleeding in women

Search NIH Clinical Center for Von Willebrand's Disease

antihemophilic factor, human Antihemophilic Factor, Human Recombinant Antihemophilic factor, porcine ANTIHEMOPHILIC FACTOR,HUMAN,METHOD M,MONOCLONAL

desmopressin Desmopressin Acetate Factor VIII

Search GEO for disease gene expression data for Von Willebrand's Disease.