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Von Willebrand Disease

  1. Reasons
  2. The prevalence
  3. Symptoms
  4. Treatment


Von Willebrand Disease

a genetic blood disease, occasionally accompanied by bleeding (similar to hemophilia). It is inherent in the autosomal dominant type of inheritance. If a person sick with the disease, his blood is missing a special protein that promotes normal clotting.

Reasons

This is the most common form of congenital disorders of the process of blood clotting, inherited disease. Often be mild, affected both men and women.

There are three types of the disease

  • type 1 is bleeding, weak and average. Hurts 75% of patients, the treatment for which often does not require
  • Type 2 insufficient activity of von Willebrand factor, bleeding, average and weak
  • Type 3 a rare form, characterized by significant bleeding.

The prevalence

The prevalence of this disease is one in 800-1000 people.

Symptoms

Symptoms depending on the form the severity of the disease, but they are sporadic.

Mild forms are accompanied by a bleeding nose, heavy menstruation in women, bleeding gums, bruising without an important reason.

Severe form characterized by profuse, prolonged bleeding, which is constantly repeated, forming a hematoma. Sometimes in the urine is also visible admixture of blood.

Bleeding the gastrointestinal tract and the fallopian is the most severe.

Treatment

Diagnosis through studies of genetic testing, measuring the time of coagulation, the analysis on the calculation of the activity of von Willebrand factor.

Therapy is carried out only by special preparations that contain von Willebrand factor. This treatment is always prescribed by a doctor taking into account individual peculiarities of the organism, but there are General guidelines.

The mild form of the disease, appoint special preparations of plasma to prevent bleeding (surgery, childbirth). In case of insufficiency of plasma, is introduced desmopressin (but it is dangerous because is accompanied by an incredibly fast addictive).

In severe forms you cannot take anticoagulant drugs.