Von Willebrand's Disease: Causes, Symptoms and Treatment

Von Willebrand disease( BV) is a hereditary pathology in which blood clotting is impaired.Clinically, it looks like hemophilia, but bleeding with it is longer.The disease is transmitted from parents to children every generation and is more common in women.On average, every thousandth inhabitant of the Earth suffers from it.

Table of Contents: Reasons for Manifesting Willebrand's Disease Diagnosis Treatment and Prevention

Reasons for

One of the parts of the human hemostatic system is the von Willebrand factor( PV), which performs two main functions:

• launches adhesion) Platelets to the site of damage to the blood vessel;
• stabilizes the coagulation factor VIII circulating in the blood.Various genetic disorders cause a defect in the synthesis of von Willebrand factor, as a result of which it is produced in insufficient quantities( in some cases, its synthesis is not possible at all).

A variant of the disease is also possible, in which the amount of PV is optimal, but the protein itself is defective and can not perform its functions.As a result, VF deficiency affects according to different data from 0.1 to 1% of the population.However, often this disease proceeds easily and can be at all not diagnosed.

Diseases of von Willebrand

For the first time, pathology is usually detected in early childhood, often in the second year of the child's life.Suspicion of von Willebrand's disease is caused by subcutaneous hemorrhages, bleeding from the nose and mouth.In severe cases without a concomitant ulcerative background, gastrointestinal bleeding occurs( mainly characteristic of the elderly).In women, the disease manifests itself in menorrhagia and postnatal metrorrhagia.

Labor in women with von Willebrand disease is sometimes a serious threat to their health due to the risk of copious bleeding.However, in most cases, during pregnancy, the synthesis of PV is normal, gradually coming back to normal, however, in the postpartum period, its concentration drops for several days.Therefore, such women should be observed at the gynecologist for at least 3 weeks.

BV flows cyclically, with periods of bleeding, followed by remissions of varying duration.The severity of the course varies widely, even among members of the same family, most often patients suffer from mild or moderate-to-severe pathology.

A diagnostic finding is a low-symptom type of disease with rare nasal bleeding.

Diagnosis

The family history and laboratory data play a leading role in the diagnosis.When a thorough survey reveals the hereditary nature of the pathology, which, together with hemorrhagic syndrome, can give the physician the necessary information to establish a correct preliminary diagnosis.

Laboratory tests can reliably detect Willebrand's disease. For this, the following tests are used:

• bleeding time for Ivy in the Shitikova modification - increase by more than 12 minutes;
• activity of factor VIII( norm 58-160%);
• activity of antigen PV( norm 55-165%);
• activity of ristocetin cofactor( norm 54-153%);
• multimeric analysis.

Changes in these indicators convincingly prove the existence of defects in the coagulation system associated with damage or deficiency of von Willebrand factor.

Treatment and prevention

People with BV are treated by hematologists.Because of the hereditary nature of the disease, it is impossible to cope with it completely, therefore, doctors struggle only with its consequences.The only effective method of treating this pathology is to stop bleeding.

For small superficial injuries, enough pressure bandages or applications of tampons with a hemostatic sponge.Severe forms of bleeding are treated with intravenous infusion of drugs containing von Willebrand factor - a substance that is absent in the patient's blood.Desmopressin - a synthesized analogue of vasopressin, as well as derivatives of aminocaproic and tranexamic acids and hormonal oral contraceptives possesses a good effect.

To prevent the occurrence of this disease is impossible, since it is inherited by the child from the sick parent.You can only reduce the risk of bleeding by preventing injury, prohibiting the use of acetylsalicylic acid in any dose and dosage form.Operations are carried out only on vital indications.Pregnant women with BV should be registered with a hematologist.

Bozbey Gennady Andreevich, ambulance doctor