Disease of von Willebrand
In 1926, Willebrand first observed on the Åland Islands a family whose members suffered from a peculiar haemorrhagic diathesis similar to hemophilia. This diathesis is transmitted according to the autosomal dominant type of inheritance. Among hereditary hemorrhagic diathesis, von Willebrand's disease ranks 3rd in frequency after haemophilia A. On average, 2 children with this disease are born per 100 000 population. At the heart of the development of von Willebrand's disease is the disruption of the formation in the body of the main component of blood coagulation factor VIII, referred to in the literature as von Willebrand factor( VIII: PV).This leads to a violation of blood clotting and the appearance of bleeding.
The severity of bleeding in von Willebrand disease varies from mild forms to extremely severe variants. A mild variant of von Willebrand's disease occurs with rare nasal bleeding and small bleeding in the skin of the spotted-bluish type. The severe course of the disease is characterized by very frequent, prolonged and profuse bleeding of different locations( uterine, nasal, gastrointestinal, renal), formation of hematomas and large bleeding in soft tissues and internal organs. Sometimes there are hemorrhages in the joints( hemarthrosis).
Intensity of nasal, gastrointestinal and uterine bleeding does not always correspond to the degree of impairment of the ability of blood to clot. In particular, sometimes against the background of moderate violations persistently repeated catastrophic bleeding of any one localization. In such cases, most likely, there are some additional local vascular disorders that cause bleeding. For their detection, a thorough additional examination of the mucous membranes of the nose, throat and pharynx, mouth, stomach and intestines is necessary. To this end, the following research methods are used in hospitals: rhinoscopy, laryngoscopy, fibroduodenalgastroscopy, colonoscopy and others. At the same time, on the mucous membranes both in the bleeding areas and outside them, superficially located enlarged and convoluted blood vessels 1-2 mm in diameter( telangiectasia), easily giving hard to
stop bleeding, are often found. It is known from the literature that such vascular formations are the cause of recurrent gastrointestinal bleeding. They are especially dangerous in Willebrand's disease, when the main mechanisms of stopping bleeding are violated. Observations show that against the background of such a lesion of blood vessels there is an erosive-hemorrhagic gastritis with massive recurring gastric bleeding.
Von Willebrand disease is often combined with various dysplasias( underdevelopment) of connective tissue, and consequently, of all organs in which this tissue enters. In patients with this disease, the prolapse of the valves of the mitral and other valvular valves is often detected. This disorder is often diagnosed without additional research methods like rheumatic heart disease. There may be a combination of von Willebrand disease with increased skin extensibility, weakness of the ligamentous apparatus, which leads to frequent and habitual dislocations, joint loosening. Less often, the disease can be accompanied by Marfan syndrome. Bleeding due to von Willebrand disease, in addition to perseverance and a stable connection with one single organ, is difficult to treat with transfusion of plasma and cryoprecipitate and is clearly amplified after traumatic local effects( eg, tamponade, moxibustion).
Contradictory data on how to tolerate women suffering from von Willebrand disease, pregnancy and childbirth. The reports of several authors indicate the fact that the level of coagulation factors during pregnancy increases, reaching a maximum in childbirth. In this regard, bleeding stops or sharply weakens. However, in other women such improvement is not observed, which leads to an increased risk of developing severe bleeding in childbirth.
Severity of bleeding during surgical interventions and injuries in von Willebrand disease is closely related to the level of factor VIII in the plasma, i.e., the lower the level of coagulation factors, the more intense and massive the bleeding, and vice versa.
The main method of treatment that provides temporary normalization of all disturbed mechanisms of blood clotting is transfusion therapy - the administration of blood products containing a complex of coagulation factor VIII, including von Willebrand factor( VIII: PV).For this purpose, antihemophilic plasma and cryoprecipitate are most often used. Before surgery, intravenous infusions of blood products begin 2-4 days before surgery, and at birth - at the very beginning of labor. Substitution therapy with blood products is carried out in the event of the development of prolonged bleeding of any location, although with uterine bleeding such treatment is not always effective.
For mild and moderate diseases, treatment is carried out with e-aminocaproic acid( promotes blood coagulation), which is prescribed for all bleeding from small vessels, including menstrual bleeding from the first day of the menstrual cycle to the end of menstruation. If inefficiency of e-aminocaproic acid is prescribed, synthetic contraceptive drugs - infecundin, mestranol. Joint use of e-aminocaproic acid, contraceptives and cryoprecipitate should be avoided. This is associated with a high probability of complications in the form of formation of vascular thrombi and DIC syndrome.
Nasal bleeding is stopped in the same way as in hemophilia.
Currently, the plasma fraction 0-1, cryoprecipitate and fresh plasma are used to treat and prevent bleeding, while preparing and performing surgical procedures.
In some cases, if there is such a need, surgical treatment is carried out - vasoconstriction, removal of part of the vessel, etc.