Von Willebrand's Disease: Types, Symptoms, Diagnosis 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.

Contents: Causes vWD Symptoms Willebrand Diagnostics vWD Treatment &

prevention reasons vWD

One part of hemostasis human body system is the von Willebrand factor( vWF), performs two basic functions:

• triggers a mechanism of adhesion( adherence) of platelets to the site of damage to the blood vessel;
• stabilizes the coagulation factor VIII circulating in the blood.

various genetic abnormalities of vWF are responsible for synthesis defect, whereby it is produced in insufficient quantities( in some cases, it is generally impossible synthesis).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.

symptoms of von Willebrand disease

first detected pathology is usually in early childhood, often already in the second year of 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 Willebrand disease sometimes pose 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.

Diagnostic finding is considered a low-symptom type of the disease with rare nasal bleeding.

Diagnosis of von Willebrand disease

When the diagnosis of the leading role played by family history data and laboratory values.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. used for this purpose the following tests:

• duration of bleeding Ivy modification Shitikova - an increase of more than 12 minutes;
• activity of factor VIII( norm 58-160%);
• activity of antigen PV( norm 55-165%);
• activity of cofactor ristocetin( 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 suffering from BV, hematologists treat.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 oral contraceptives possesses a good effect.

To prevent the occurrence of this disease is impossible, because 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 conducted only for vital indications.Pregnant women with BV should be registered with a hematologist.

Bozbey Gennady, ambulance doctor