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Von Willebrand disease


Disease: Von Willebrand disease Von Willebrand disease
Category: Blood diseases & tumors

Disease Definition:

Von Willebrand disease is the most common inherited bleeding condition, which involves extended or excessive bleeding. One of the important components in the blood clotting process is the protein called von Willebrand factor. When this protein becomes impaired or is deficient, von Willebrand disease develops.


Forming blood clots and stopping bleeding when being cut usually takes longer for people with this disease. Preventing or stopping the bleeding episodes, usually with medications, is what treatment for von Willebrand diseases focuses on. People with this disease can lead normal and healthy lives in case they receive the right kind of treatment.

Work Group:

Symptoms, Causes


The signs and usually mild and they may be absent altogether in numerous people suffering from von Willebrand disease (vWD). The intensity of the signs varies from one person to another in case they do occur. Doctors usually don’t notice von Willebrand disease in the diagnosis process in case the condition is mild. Although it might be present at only moderate levels, but abnormal bleeding is the most common sign of von Willebrand disease. The abnormal bleeding related to von Willebrand disease might occur as:


  • Increased menstrual flow
  • Recurrent and prolonged nosebleeds
  • Blood in the stool or urine
  • Bleeding from the gums
  • Excessive bleeding from a cut or following a tooth extraction or other dental process
  • People suffering from von Willebrand disease might also experience bruises with lumps that form underneath the skin, and easy bruising.


Only after extensive bleeding due to serious trauma or a surgical procedure may some people notice that they have a bleeding disorder.


Signs and symptoms in women:

In women, the primary sign of von Willebrand disease is heavy menstrual bleeding. But because many women are not overly concerned about their prolonged or heavy menstrual bleeding, many cases of von Willebrand disease go undiagnosed. Heavy menstrual bleeding might be overlooked as a possible indicator of von Willebrand disease.


Listed below are some of the signs and symptoms of an abnormally heavy period that may indicate von Willebrand disease:


  • The need to change the menstrual pad or tampon more frequently than hourly
  • The presence of blood clots in the menstrual flow greater than 1 inch (2.5 centimeters) in diameter
  • Menstrual bleeding lasting longer than one week
  • Symptoms of anemia including fatigue, tiredness or shortness of breath
  • The need to use double sanitary protection to control menstrual flow


CLASSIFICATIONS OF VON WILLEBRAND DISEASE:  Von Willebrand disease has three major types:


Type 1:

This is the most common form and may have mild signs. They seem to occur in people with only modest declines in the blood levels of both von Willebrand factor and factor VIII, a substance that’s carried in the person’s blood by von Willebrand factor that helps stimulate clotting.


Type 2:

Signs tend to be more intense in this type, which has several subtypes. The von Willebrand factor that is present doesn’t function properly in this type.


Type 3:

Von Willebrand factor is lacking altogether and people have low levels of factor VIII in this rare type. Signs and symptoms might be severe in intensity, and there may be bleeding into the joints and muscles. 


A person should consult the doctor in the case of experiencing extended or severe bleeding. Von willebrand disease increases the likelihood of post-surgical bleeding; because of this, a person with this disease should make sure to tell his/her doctor or dentist about it. This goes for any type of surgery, including dental procedures. The person might also consider wearing a medical ID bracelet, noting that they have von Willebrand disease, in case he/she had an accident and is taken to an emergency room.


A defect in the gene controlling von Willebrand factor is the cause of von Willebrand disease. Platelets, which are small blood cells, can’t stick together normally and they can’t attach themselves normally to the blood vessel walls when an injury occurs in case the von Willebrand factor is scarce or when it doesn’t function normally due to structural abnormalities. Persistent uncontrolled bleeding and an interference with the clotting process will result from this.


The leading risk factor of von Willebrand disease is family history. A child may inherit the abnormal gene from a parent. In case a person has the gene for von Willebrand disease, they have a 50 % chance of transmitting this gene to their offspring because most cases are inherited in the autosomal dominant pattern. In case a child inherits the gene from both of the parents, he/she will inherit the most severe form of the condition, namely, type 3. However, race doesn’t seem to play a role in the disease. Males and females are equally affected by von Willebrand disease.



Von Willebrand disease may cause some of these complications:



Iron deficiency anemia may develop in women experiencing heavy menstrual bleeding.


Swelling and pain:

Swelling and severe pain can result from an abnormal bleeding that occurs in the joints or soft tissue.


Death from Bleeding:

The abnormal bleeding may become life-threatening and need emergency medical attention in case it can’t be controlled.


Von Willebrand disease can be effectively treated, despite the fact that it is a lifelong condition that doesn’t have a cure. Depending on the kind and severity of the disorder, as well as the person’s response to previous therapy and other medications that he/she might be taking, the treatment method may vary. Some of the methods that are commonly used in the treatment of von Willebrand disease are:


Desmopressin (DDAVP):

This a synthetic hormone, similar to the natural hormone vasopressin that controls bleeding by inducing the person’s body to release more von Willebrand factor already stored in the lining of the blood vessels, thereby enhancing factor VIII levels. This medication is administered by injection into a vein or, more commonly, through a nasal spray. People with type 1 and those with some subtypes of type 2 of the disease benefit most from DDAVP. Most doctors consider DDAVP the initial treatment in managing von Willebrand disease. In order to control excessive bleeding, some women use the nasal spray at the beginning of their menstrual periods. The person could also use it before a minor surgical procedure.


Replacement therapies:

Infusions of prepared doses of concentrated blood clotting factors that contain von Willebrand factor and factor VIII are involved in these therapies. The patient may be recommended these therapies in case they need treatment for more-severe forms of the disease or if DDAVP isn’t effective, because these therapies could be beneficial in all types of the disease.



The heavy bleeding during menstrual periods could be controlled by birth control pills. The levels of von Willebrand factor and activity of factor VIII are increased by the estrogen hormones that are present in these medications. On the other hand, placing a progesterone-containing contraceptive device in the uterus could be another option.


Antifibrinolytic or clot-stabilizing medications:

The breakdown of clotting factors could be slowed down by these medications, such as tranexamic acid and aminocaproic acid. This could stop bleeding by helping keep a clot in place once it has formed. Before or after a surgical procedure or tooth extraction, the patient may be prescribed these medications.


Fibrin sealants:

To reduce bleeding, these substances that are applied like a glue using syringes are put directly on a cut.


Treatment is only recommended when the person is undergoing surgery or dental extractions, or when experiencing trauma (For instance, in an automobile accident) in case the condition is mild.


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