Von Willebrand Disease

December 22 22:05 2019 Print This Article

Von Willebrand’s factor is a glycoprotein produced both by platelets (the blood cells involved in clotting) and by the cells lining blood vessels. First described by Erik Adolf von Willebrand in 1926, VWD is a congenital bleeding disorder characterized by a lifelong tendency toward easy bruising, frequent epistaxis, and menorrhagia. This is a protein in blood which is necessary for proper blood coagulation, or clotting.

Unlike hemophilia, which usually affects only guys, VWD affects both guys and girls. It is the most common inherited bleeding disorder, affecting as much as 1% of the population or more. This clotting protein is called von Willebrand factor. It is the most common hereditary bleeding disorder. When some of this factor is missing or defective it can cause prolonged bleeding after an injury or accident. If the von Willebrand factor is working properly, it helps your blood clot.

There are three types of von Willebrand’s disease. In fact, it is not a single disease, but a family of related diseases. Normally, bleeding occurs when a blood vessel is cut or torn. Platelets (small cells that float in the blood) plug the hole in the blood vessel to stop the flow of blood. A substance in the blood known as von Willebrand factor helps platelets stick to damaged blood vessels.

Von Willebrand factor acts like glue that helps the platelets stick together. Von Willebrand factor is also a carrier of clotting factor VIII, another protein that helps your blood to clot. People with von Willebrand Disease are unable to make this plug because they do not have enough von Willebrand Factor or their factor is abnormal. If there is too little von Willebrand factor or the factor is defective, platelets do not gather properly when a blood vessel is injured.

Causes of Von Willebrand Disease

The comman causes of Von Willebrand Disease include the following:

  • Von Willebrand disease is caused by a deficiency of von Willebrand factor.
  • Hypothyroidism.
  • In some cases, VWD is believed to result from other pathologic processes; however, because of the relatively high prevalence of VWD, its concomitant occurrence with other disease states may be coincidental.
  • Wilms tumor.
  • Congenital heart disease.
  • Most cases are mild, and bleeding may occur after a surgical procedure or tooth extraction.
  • Von Willebrand’s disease is passed down through families. It is the most common bleeding disorder present at birth (congenital).
  • The condition is worsened by the use of aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs).

Symptoms of Von Willebrand Disease

Some sign and symptoms related to Von Willebrand Disease are as follows:

  • Bleeding of the gums.
  • Blood in the stool.
  • Abnormal menstrual bleeding.
  • Nose bleeds.
  • Gingival bleeding.
  • Bruising.
  • Skin rash.
  • Postpartum bleeding.
  • Easy bruising.
  • Frequent or prolonged nosebleeds.
  • Blood in the urine.
  • Heavy menstrual flow.

Treatment of Von Willebrand Disease

Here is list of the methods for treating Von Willebrand Disease:

  • Medications such as desamino-8-arginine vasopressin (DDAVP) can be given to raise the levels of von Willebrand factor, which will reduce the tendency toward bleeding.
  • Birth control pills-may be used to control heavy menstrual periods in women with type 1 VWD.
  • Blood plasma or certain factor VIII preparations may also be used to decrease bleeding.
  • Antifibrinolytic medicine (often, Amicar)-can be used for bleeding in the nose or mouth.
  • Fibrin glue is medicine placed directly on a wound to stop the bleeding.
  • Replacement therapy is the injection of a concentrate of von Willebrand factor and factor VIII.