Willebrand ‘s disease
CAUSES OF WILLEBRAND’S DISEASE AND CLASSIFICATION
Willebrand’s disease is a rare blood disease characterized by episodic bleeding. Its cause lies in blood clotting disorders, which is provoked by insufficient activity of certain substances in the blood serum (the so-called Willebrand factor).
Willebrand’s disease is transmitted exclusively by inheritance and is congenital. It often happens that the disease of the first type remains unnoticed throughout the patient’s life. The disease is mildly progressive, the change of severity forms is extremely rare. The gender of the patient does not affect any role, both men and women are equally susceptible to the disease.
This disease is usually divided into three main types.
In Willebrand’s disease of the first type, there is a decrease in the content of Willebrand factor in blood serum. The ability to coagulate blood is preserved, but the process takes a long time. With this type of disease, minor hemorrhages may occur, but bleeding can be stopped by classical methods. In some cases, patients do not need additional treatment. This form of the disease is most common.
The second type of disease means that the functions of the Willebrand factor are impaired while maintaining its quantitative indicators at the proper level. Bleeding in this form of the disease is classified as mild and moderate.
The third type of the disease is characterized by the complete absence of the Willebrand factor in the blood serum or an extremely small amount of it. Often leads to the development of anemia, bleeding is classified as severe. This is the rarest form of this ailment.
SYMPTOMS OF WILLEBRAND’S DISEASE
The symptoms of Willebrand ‘s disease include the following manifestations:
spontaneous nosebleeds;
bleeding gums;
subcutaneous hemorrhages;
the formation of bruises for no apparent reason or with little physical impact;
the presence of blood in the secretions – urine and feces take on a dark color;
hemorrhages in muscles, joints, internal organs (rarely);
severe bleeding during menstruation in women;
heavy bleeding from injuries, cuts, surgical interventions.
Diagnosis of this disease begins with the examination and collection of patient complaints by a hematologist. It is necessary to establish whether there are any patients with this disease in the anamnesis among the patient’s relatives. Next, an examination is carried out aimed at identifying the amount of Willebrand factor in the blood serum and the rate of coagulation of a drop of the patient’s blood is measured. It is important to differentiate this disease from hemophilia at the stage of diagnosis.
PREVENTION AND TREATMENT OF WILLEBRAND’S DISEASE
The type of treatment for Willebrand’s disease depends on the form and severity of the disease. In some cases, the disease does not require specific treatment, the patient is only given recommendations for prevention. These include:
refusal or minimization of nonsteroidal anti-inflammatory drugs;
refusal to take drugs that promote blood thinning;
avoiding injuries and cuts.
With forms of the disease that require specific treatment, it is completely prohibited to take nonsteroidal anti-inflammatory drugs. Assigned:
substitution therapy with drugs containing Willebrand factor;
drugs that help stop bleeding;
in some cases, women are prescribed hormone therapy (at the time of menstruation).
Also, patients suffering from this ailment must necessarily warn a doctor (including a dentist) about the presence of the disease if any surgical intervention is planned.
