Hematology and Blood Disorders

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Commentary - Hematology and Blood Disorders (2021) Volume 4, Issue 6

Von willebrand disease: A blood disorder.

Philip Assari*

Department of Hematology, Saint Joseph University, Beirut, Lebanon

Corresponding Author:
Philip Assari
Department of Hematology
Saint Joseph University
Beirut
Lebanon
E-mail: [email protected]

Accepted date: December 29 , 2021

Citation: Assari P. Von willebrand disease: A blood disorder. Hematol Blood Disord. 2021; 4(6): 5.

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Description

Von Will disease is a chronic clotting illness in which your blood does not clot properly. The condition is passed down through the generations, with the majority of persons inheriting it from one or both parents. Many proteins in blood assist the blood clot when it is needed. Von Willebrand Factor is one of these proteins (VWF). VWD patients either have a low level of VWF in their blood or the VWF protein does not function properly.

Types of VWD

Type 1: The most frequent and mildest form of VWD, in which a person's VWF levels are lower than usual. In addition, a person with Type 1 VWD may have low levels of factor VIII (8), a blood-clotting protein. Type 1 VWD affects about 85% of persons who are treated for it.

Type 2: In this type of VWD, the body produces normal amounts of VWF, but the factor does not function properly. Depending on the exact difficulty with the person's VWF, Type 2 is further divided into four subtypes-2A, 2B, 2M, and 2N. Because each subtype requires a particular treatment approach, it's critical that a person understands which subtype he or she belongs to.

Type 3: This is the most severe form of VWD, characterised by a lack of VWF and low amounts of factor VIII. This is the most uncommon kind of VWD. Type 3 affects only 3% of patients with VWD.

Mucocutaneous hemorrhage is a common symptom of VWD. VWD is caused by a defect or lack of Von Willebrand Factor (VWF), a big protein with several components. VWF binds to clotting factor VIII in the bloodstream and prevents it from being destroyed. VWF also aids platelet adherence to the insides of damaged blood vessels. This causes a stable blood clot to form, plugging the wounded blood artery and stopping the bleeding. An individual may have trouble creating a blood clot if there is inadequate VWF or if it is faulty. The majority of those who are affected have VWD type 1, which is a very mild version of the disease that is not recognised until adulthood.

During infancy or early childhood, a tiny number of these people may experience persistent bleeding. Nosebleeds, gum bleeding, and easy bruising are all possible symptoms. Heavy menstruation is common in women with VWD. Affected people are more likely to bleed freely after an injury, childbirth, or surgery. The stomach and intestines can both bleed, but this is less usual.

Treatment for von Willebrand disease is determined by the severity of the problem. The goal of all treatment is to keep episodes of severe bleeding at bay. Desmopressin is a hormone that increases the amount of Von Willebrand Factor (VWF) in the bloodstream. It is breathed through the nose or injected into a vein. The most prevalent treatment for von Willebrand disease is this therapy. VWF infusions: Infusions of VWF are required for bleeding event and, more frequently, surgical procedures, depending on the kind of von Willebrand disease. To maintain normal levels of the VWF protein in their blood, some individuals with severe forms of von Willebrand disease undergo regular VWF infusions or preventative medication. Antifibrinolytics are medications that prevent blood clots from forming.

Von Willebrand disease is a chronic illness. For the most part, the condition does not prevent people from leading busy lifestyles. It is usually only treated after a serious accident or if surgery is required. The best method to ensure that von Willebrand disease does not interfere with living a healthy life is to seek treatment when needed and to see your doctor on a frequent basis.

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