What is von Willebrand disease causes diagnosis how to treat

With normal homeostasis, a bleeding vessel is clogged by the necessary enzymes, forming a site with clotted blood. A patient with a disease undergoes a complete disruption of this process and the lack of the body’s ability to coagulate on its own.

The main symptom of the disease is bleeding, which is difficult to stop. They have different localization, severity, intensity.

The cause of such bleeding can be any injury, surgery, visiting a dentist. Exacerbation of the disease is accompanied by poor health, weakness, dizziness, nausea and fainting. There is a direct relationship between the nature of the disease and its intensity of bleeding.

In childhood, the clinical picture that accompanies von Willebrand disease has a burdened etiology. Due to underdeveloped immunity, exacerbations of the disease occur after each acute respiratory viral infection, acute respiratory infections. Under the influence of infectious agents, the walls of the vessels become vulnerable, acquire increased insight, causing various bleeding. The disease refers to incurable pathologies. During life, the period of absence of the clinic is replaced by exacerbations.

Symptoms of von Willebrand disease:

  • Abundant and prolonged menstruation in women. Often requiring medical attention to stop bleeding.
  • Impurities of blood after the act of defecation, in the absence of deviations in the gastrointestinal tract.
  • Multiple formations on the skin of a hemorrhagic nature.
  • Frequent and prolonged bleeding after simple surgical procedures, tooth extraction.
  • Chronic severe anemia.
  • Unreasonable bleeding, which lasts more than 10 minutes and requires urgent medical attention.
  • Self-formed hematomas and bruises. Or after minor injuries, the appearance of blood and bleeding.
  • The manifestation of prolonged bleeding after the acquisition of any injury. Relapse occurs after a week.

In addition to all of the above, von Willebrand disease is often more severe. It forms bleeding organs, mucous membranes, affects joints.

According to the recommendations of the Scientific and Standardization Committee (SSC) under the International Society for Thrombosis and Hemostasis, von Willebrand disease is classified as follows:

  • Type I – a decrease in the synthesis of von Willebrand factor and related antigen. In type I blood plasma, all multimers of the factor are present. It occurs in 55–75% of cases;
  • Type II – a sharp decrease in the formation and content in the blood of the most active large multimers of von Willebrand factor, the predominance of small multimers with low hemostatic activity;
  • Type III – the almost complete absence of von Willebrand factor, is extremely rare (in 1-3% of cases).

Depending on the characteristics of phenotype II, the type of disease is divided into several subtypes:

  • IIA — qualitative factor defects with a decrease in vWF-dependent platelet adhesion and an isolated deficiency of high molecular weight multimers;
  • IIB – qualitative defects of von Willebrand factor with increased affinity for the platelet GP-Ib receptor;
  • IIN – violation of the ability of von Willebrand factor to bind to coagulation factor VIII;
  • IIM – the presence of vWF-defects against the background of the lack of deficiency of multimers.

A number of factors affect the level of von Willebrand factor in the blood:

  • age (level increases as you grow older);
  • floor;
  • ethnicity (level higher among Africans and African Americans);
  • blood group (the half-life of the factor in individuals with blood group I is approximately 1/4 less than in carriers of other groups);
  • hormonal background (decreased with hypothyroidism, increases during pregnancy).

Von Willebrand disease is transmitted genetically from generation to generation. Pathology is quite rare – in one of 800-1000 people, but often proceeds in a mild form. The likelihood of an ailment in people of both sexes is the same.

In a healthy body, in case of bleeding, blood cells called platelets rush to the site of damage to the vessel and stick together, clogging the “hole”.

In the case of von Willebrand disease, the normal coagulation process is impaired due to the complete absence, defect, or lack of a special protein that is directly involved in the formation of a thrombus (that is, “sticking” of platelet aggregation to the damaged area). This protein is called the von Willebrand factor.

Etiological (causal) factors of pathology are:

  • burdened heredity;
  • congenital coagulation defects;
  • lymphoproliferative, autoimmune diseases.

There are several types of this disease:

  • Type 1. It is characterized by a deficiency of von Willebrand factor, which is manifested by small or moderate bleeding, respectively, a lack of this protein. Among patients suffering from this ailment, 80% of this disease is detected (more often by accident). No therapy needed.
  • Type 2. There is a von Willebrand factor, but protein, due to a defect, does not fulfill its functions. Most often, bleeding is insignificant, but a severe course is also possible.
  • Type 3. It occurs in 5% of cases. It is characterized by the complete absence or presence of an extremely small amount of von Willebrand factor, as a result of which patients experience massive bleeding during surgery or as a result of injuries. Anemia is often observed.

In addition, a platelet type of disease is distinguished, in which, due to a gene mutation, the sensitivity of the platelet receptor to high molecular weight multimers of von Willebrand protein increases.

There is also acquired von Willebrand disease, which occurs in patients suffering from lymphoproliferative and autoimmune pathologies. This type of disease develops as a result of the appearance of a specific inhibitor of von Willebrand factor.

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Risk groups

The risk groups include patients:

  • having burdened heredity (genetic predisposition);
  • suffering from clotting disorders due to various pathologies and injuries.

Main symptoms

The most specific symptom for von Willebrand disease (ICD-10 – D68.0) is bleeding from the nose, internal organs, gums. Moreover, bleeding can be either insignificant or massive, and proceeds more often according to the microcirculatory type.

Patients with a significant platelet protein deficiency, bleeding (uterine, gingival, nasal) are prolonged and profuse, in addition, hemorrhages in the joints and muscles often occur. Prolonged bleeding can also occur when teeth are removed, operations and injuries.

Other manifestations

Hemorrhages in muscle tissue and subcutaneous tissue (hematomas) occur with this pathology mainly as a result of injuries in patients with severe illness.

Hemorrhagic syndrome in von Willebrand disease is not a constant sign of the disease. The periods of its exacerbation alternate with an almost complete absence of hemorrhages.

In some cases, von Willebrand disease is combined with mesenchymal dystrophy. At the same time, patients exhibit excessive skin extensibility, prolapse of valve flaps in the heart, increased joint mobility with ligament weakness.

Due to autosomal inheritance, the disease affects women and men with the same frequency. However, due to physiological characteristics (reproductive function), hemorrhagic symptoms in women appear more often. So, 65% of patients suffer from menorrhagia. Heavy recurrent uterine bleeding, as a rule, lasts more than 10 days, complicated by posthemorrhagic anemia.

Bleeding from the digestive organs (duodenum, stomach) is not a prevailing symptom. Such bleeding often occurs as a result of the use of drugs that affect platelet aggregation (aspirin, NSAIDs). In addition, latent ulcers of the duodenum and stomach, hemorrhoids or erosive gastritis can be a source of bleeding.

Probably the appearance of hematuria (the presence of blood impurities in the urine) or the presence of blood impregnations in the feces.

In patients suffering from this ailment, profuse, prolonged bleeding that occurs during operations or childbirth is possible. Childbirth in patients with von Willebrand pathology is associated with a high risk of significant blood loss.

Moreover, in the majority of patients suffering from mild or moderate form of the disease, during pregnancy, the concentration of von Willebrand protein increases by 3 times, reaching almost normal levels, but after childbirth comes to its previous level.

The most rare symptom of the disease, characteristic of the 3rd type, is hemarthrosis. In acute hemarthrosis, there is significant soreness associated with an increase in intraarticular pressure.

At the same time, the volume of the joint is increased, and the skin in its area is hot and hyperemic. When the cause of hemarthrosis was an injury, it is necessary to exclude associated damage (separation of the condyle and others).

Chronic synovitis is a consequence of recurrent hemarthrosis. In this case, hypertrophied synovial membrane acts as the main source of articular hemorrhage.

Acute synovitis occurs with frequent relapses of hemarthrosis, occurring even with transfusions of the necessary coagulation factor, due to the presence of inflammation in the synovial membrane.

Chronic synovitis, in the case of a destroyed joint capsule, can be painless.

The pathology of von Willebrand, unlike hemophilia, is not accompanied by the formation of deforming osteoarthrosis.

Hemorrhages in the membranes and directly into the tissue of the spinal cord / brain in the presence of von Willebrand disease are the result of various injuries. In rare cases, the causative factor may be a hypertensive crisis or the use of drugs that adversely affect the hemostatic functions of platelets, such as butadione, aspirin, and so on.

Willebrand’s disease in children is most often manifested by bruises on the skin, nose and gingival bleeding. The most severe course of the disease is noted immediately after or during infectious pathologies. In this case, the mechanism of such spontaneous bleeding of the diapedetic type is most likely an increase in vascular permeability due to the infectious process.

Manifestations of BV

Signals of the disease are nonspecific, many do not pay attention to them. Bleeding is more common in children. In adults, they are less often and not so intense, but this happens subject to all the recommendations of the attending physician. In some cases, exacerbations of the disease are found that are dangerous to the patient’s life.

The following clinical manifestations are observed:

  • The formation of hematomas under the skin, even with a slight physical impact.
  • Unreasonable nosebleeds lasting more than 10 minutes.
  • The manifestation of bleeding after surgery, injuries (more than a week).
  • Prolonged heavy periods, accompanied by severe pain, general weakness, often cause fainting.
  • Pallor of the skin.
  • Hemorrhagic syndrome with the formation of a rash.
  • There is blood in the feces, diseases of the gastrointestinal tract are absent. In such cases, internal bleeding, which can cause the death of a person, is not excluded.
  • Severe anemia.

If nosebleeds are observed in a child, this may be due to congenital pathology, it is necessary to seek medical help.

During pregnancy, the disease may not manifest itself, but in the birth and postpartum period can cause severe blood loss. It requires special control by doctors.

Signs of von Willebrand disease are different from other pathologies, they occur unexpectedly, do not have constancy, and their manifestations are not homogeneous. This disease is called a chameleon.

Willebrand disease can manifest itself:

  • In a newborn baby after birth;
  • Do not appear for quite a long time;
  • Manifest yourself suddenly abundant leakage of blood after a minor injury;
  • Show yourself after a previous infectious or viral disease.

This pathology can occur in a mild course of the course, which does not represent a mortal danger, as well as in a severe (life-threatening) degree of the course of the disease.

Symptoms of a mild degree of von Willebrand disease:

  • Epistaxis that occurs systematically over a short period of time;
  • Heavy bleeding during menstruation in women;
  • Prolonged bleeding from a minor cut or minor injury;
  • Petechiae on the skin;
  • Post-traumatic hemorrhages.

The severe stage of the course of von Willebrand disease manifests itself in vitally dangerous symptoms:

  • Dysuria appears pain in the lumbar spine and blood in urine;
  • Large localization of the hematoma after a minor bruise, squeezes large diameter arteries, which causes pain;
  • Heavy bleeding after trauma, as well as internal post-traumatic blood flow;
  • Severe bleeding during surgery in the body;
  • Bleeding after tooth extraction surgery;
  • Recurrent bleeding from the sinuses and blood from the gums;
  • Prolonged bleeding after cuts on the skin, which can cause a fainting state, dizziness;
  • Heavy monthly uterine bleeding, provoke a deficiency in the composition of the blood of iron, which manifests itself in anemia;
  • Hemarthrosis disease is the swelling of the affected joint, painful conditions in the joints, as well as an extensive subcutaneous hematoma;
  • Abundant leakage of blood of their nasopharynx, as well as pharynx – leads to pathology bronchial obstruction;
  • Cerebral hemorrhage affects the centers of the entire nervous system and leads to death.

In the second type, it can occur only in the case of an abnormality in the structure of platelets, as well as in the formation of hemarthrosis.

If the von Willebrand factor is lower (its deficiency), then an anomaly in the structure of the platelet molecule joins, then the pathology has such characteristic signs:

  • Severe nasopharyngeal bleeding;
  • Bleeding inside muscle tissue and their manifestations in the form of a hematoma;
  • Bleeding of internal organs: stomach, intestines (with injuries);
  • Damage to the pathology of the joints.

The most dangerous for human life are gastric bleeding and intestinal bleeding inside the abdominal cavity. Arteriovenous anastomoses prevent the stop of internal bleeding.

Willebrand’s disease in childhood is diagnosed and manifests up to 12 months.

Bright symptoms in infancy include:

  • Leakage of blood from the nose;
  • Blood from gums during teething;
  • Bleeding inside the stomach, as well as blood in the intestines – manifests itself in feces;
  • Blood in urine.

Vivid symptoms and signs of iron deficiency anemia are expressed – this is pallor of the skin, dizziness, fainting, signs of nausea.

This pathology is also expressed in rashes on the skin in the form of ecchymoses that are filled with blood, in bruises on the skin and in hematomas of intraarticular hemorrhage.

Bleeding begins immediately after the injury, and after it has been stopped, relapses do not recur – this is the main difference between von Willebrand disease and hemophilia.

Symptoms of the disease are not too extensive and include common signs characteristic of each type of pathology.

Symptoms, the appearance of which may suspect the presence of the disease:

  • the appearance of bleeding even with a slight injury to the skin;
  • bleeding in the form of hematomas under the skin;
  • uterine bleeding;
  • long periods;
  • gastric and intracranial bleeding (extremely rare and only with the III form of the disease);
  • hemarthrosis – hemorrhage in the joint cavity (also occurs infrequently and affects single joints, which does not affect their functions).

Symptoms of von Willebrand disease characteristic of a particular form of the disease:

  • I and II forms are manifested:
    • nosebleeds;
    • frequent and causeless subcutaneous hematomas;
    • prolonged bleeding of wounds after injuries / cuts;
    • uterine bleeding;
    • long periods;
    • excessive blood loss during childbirth and operations;
  • Form III is similar to hemophilia and may have its symptoms:
    • frequent subcutaneous bleeding;
    • large hematomas, very painful when pressed;
    • hemorrhages in large joints;
    • heavy bleeding from the gums, nose;
    • gastric and urological bleeding.

If there is a disease, it is important for a woman to warn the gynecologist about this. Pregnancy should be planned, so it is worth using suitable contraception methods, because pathology can become an obstacle to a happy motherhood.

Pregnant women with von Willebrand disease have a 30% risk of miscarriage. Late toxicosis is also common. Of the most serious consequences – premature detachment of the placenta with severe bleeding. In 50% of women, childbirth occurs with complications. Therefore, the expectant mother should always be under the supervision of doctors.

In the presence of pathology in relatives, it is important to examine newborns in the family. Severe forms of the disease appear in the first year of life.

The most common symptoms are:

  • spontaneous bleeding from the nose, mouth;
  • bleeding gums;
  • bleeding from the digestive tract (detected in feces).

In adolescent girls, pathology is manifested by menorrhagia (bleeding from the uterus), which is accompanied by signs of anemia (pallor of the skin, dizziness, weakness). Often, bruises, a rash on the skin, and intraarticular hematomas are observed in the disease.

The association of BV with pathologies in connective tissue

Studies by modern scientists have established the relationship of von Willebrand disease, as well as defects and abnormalities in the development of connective tissue cells.

This relationship is manifested in such violations:

  • Mitral valve prolapse and other heart valve pathologies;
  • Dislocations in the joints due to a violation in the ligaments of muscle organs;
  • Hyperelastosis of the skin;
  • Degenerative myelopathy;
  • Marfan syndrome manifests itself in cardiac pathologies, in disorders of the ocular organ, as well as elongation of skeletal bones in the body.

How close is this relationship between these two pathologies and its etiology, it is necessary to find out through research by scientists.

Genetics, using research, concluded that the pathogenesis of von Willebrand disease depends on the nature of genetic inheritance.

With homozygous genetic inheritance (from two biological parents), then von Willebrand disease proceeds in a severe clinical form, with all types and characteristics of bleeding, and quite often with complete absence of antigen f VIII in the blood plasma.

Less severe symptoms are also manifested than with hemophilia pathology, in lesions of the articular part of the body and in muscle organs.

During pregnancy and childbirth

It is impossible to predict the course of pregnancy with pathology, more often, under the influence of estrogens, the quantitative von Willebrand factor increases, the same process is observed immediately before childbirth. But it all depends on the physiological characteristics.

With type I pathology, the level of PV is normalized to an indicator of a non-pregnant woman. In type II, the factor increases, changes in coagulability are small, the pathological structure is preserved. In type III, factor VIII and PV do not exceed the norm at all or does not do much.

The time of birth and the first 24 hours after them are dangerous with primary bleeding, a day after them, up to a month and a half, there remains a risk of secondary bleeding. Therefore, women who have given birth should be monitored by a doctor and follow his recommendations.

The course of pregnancy with von Willebrand disease types No. 1 and No. 2 partially compensates for the von Willebrand factor by the time of the birth process. Although its volume is higher than normal, it is still not enough for blood coagulation at the time of delivery and in the postpartum period. Gynecologists are always afraid of sudden and heavy birth bleeding.

The most serious complication of von Willebrand disease during the period of intrauterine formation of the child is the premature detachment of the placenta with its correct intrauterine location. With normal therapy and monitoring of this pregnant woman within the walls of the hospital, the birth process takes place in a timely manner.

Sometimes pregnancy improves the course of this pathology. And quite often after the birth of a child, remission of von Willebrand disease does not occur for a very long time.

Dangers during pregnancy, at the time of birth and the postpartum period:

  • Miscarriage at the initial stage of pregnancy (1 trimester);
  • Detachment of the placenta (3 trimester);
  • At the time of birth – heavy bleeding, which is very difficult to stop;
  • 6 calendar days and up to 10 days after the hearth process – the danger of bleeding;
  • Up to 13 calendar days, the danger after cesarean section is an increased amount of bleeding.

Pregnancy with this dangerous ailment should always be planned. That is why a woman, even before her onset, should visit a gynecologist and warn the doctor about the presence of such an ailment in her history. Before planning for conception, the use of reliable contraceptives is recommended, since von Willebrand disease can often become a significant obstacle to the safe bearing and birth of a child. In addition, abortion in this disease is very dangerous for the health and life of women.

  • Nearly 30% of pregnant women with this diagnosis have a risk of miscarriage and late toxicosis.
  • 50% of women with von Willebrand disease have to deal with a complicated course of childbirth and that is why pregnant women with this ailment should be constantly monitored by specialists.
  • The most dangerous complication of such a disease during pregnancy can be premature placental abruption.
  • Often with any form of such a disease, bleeding of various intensities occurs during and after childbirth.

Pregnancy has a mixed effect on the course of von Willebrand disease. Often when pregnancy occurs under the influence of estrogen, the level of von Willebrand factor in the blood plasma rises, the same tendency can be observed before childbirth. However, these changes do not occur in all women.

According to the results of studies, there is a high frequency of spontaneous abortion in the early stages – 22-25% in women suffering from this pathology. Approximately 1/3 of women recorded bleeding in the first trimester of pregnancy.

Most women with type von Willebrand disease achieve normal (for non-pregnant) factor levels in the last trimester of pregnancy. In type II disease, the levels of factor VIII and von Willebrand factor often increase during pregnancy, but most studies have noted a minimal or no change in the level of activity of coagulation factors and the preservation of the pathological structure of multimers. In women with type III disease of von Willebrand disease, the levels of factor VIII and von Willebrand factor practically do not increase during pregnancy.

Women with von Willebrand disease have a high risk of spontaneous abortion in early pregnancy

Women with von Willebrand disease are at increased risk of primary (more than 500 ml on the first day after childbirth) and secondary postpartum hemorrhage (from 24 hours to 6 weeks) due to a rapid decrease in the activity of blood coagulation factors after childbirth.


Signs of the disease can occur with insufficient levels of EF. The norm for a blood test should be 10 mg / l.

During pregnancy, during inflammatory or infectious processes, stressful situations, with active sports, the use of hormonal drugs, the indicator is increased. In people with the first blood type – lowered.

The normative indicators for patients with blood group I will differ from those of people with the second, third, and fourth blood groups.

For the diagnosis, it is recommended to undergo three types of tests:

  • PV: antigen level (VWF test: Ag);
  • PV: on the activity of a factor in blood plasma (VWF test: Act);
  • PV VIII in the ratio: FVIII to VWF: Ag.
VWA Ag – normal
VWF Act – norm
FV III – normal
von Willebrand disease and hemophilia pathology – not present
PV is very reducedtype 3 disease
VWF Act – 1
VWF Ag – 1
type 1 disease
VWF Act, VWF Ag less than 0,70pathology type No. 2 – 2A, 2B, 2M
FV III, VWF Ag, less than 0,70type 2 disease – 2 N, as well as hemophilia pathology
(FVIII: C), (VWF: Ag) and (VWF: AC) increases.during pregnancy with type No. 1
VWF Act, VWF Ag, FV III = 0with type number 2 – 2A, 2B, 2M

Normally, the average concentration of von Willebrand factor in venous blood is 10 mg / L. In the test results, it is often indicated not the concentration of this substance, but a percentage value indicating how much the actual content in the test blood differs from the average. The norm in this case is 50 – 160%.

What is von Willebrand factor and what are its functions

Von Willebrand factor (vWF) is a large blood protein that can cause platelet activation and stabilization of factor VIII of the blood coagulation system, which is necessary to stop bleeding. Performing this role, it, after entering the bloodstream, undergoes splitting with a metalloprotease and is transformed from a macromolecule into mono- and dimers (smaller molecules).

This is one of the few proteins that carries agglutinogens of the AB0 blood group on its surface. Scientists noted that it is group affiliation that determines the level of von Willebrand factor – in people with the first group its level is minimal, and in people with the fourth group it is maximum.

vWF is produced by the endothelium (the membrane lining the lumen of blood vessels) and accumulates in the vascular bed in the form of Weibel-Paladi bodies. In addition, the synthesis of the factor is carried out by alpha granules and giant maternal platelet cells located in the red bone marrow.

In the study of vWF, attention is paid not only to its level, but also to functional activity. The von Willebrand factor performs the following functions:

  • guarantees platelet gluing (primary hemostasis) and their adhesion (gluing) to the vascular wall;
  • protects antihemophilic globulin (factor VIII) from protein C or factor Xa, stabilizes factor VII.

Treatment prognosis and complications: when disability is assigned

As for the I and II forms of the disease, the prognosis is favorable. It is noted that after puberty the symptoms are reduced.

However, a fatal outcome is also possible – when the patient suffers from a third form of pathology and does not have access to qualified medical care during heavy bleeding.

Complications of the disease include:

  • severe posthemorrhagic anemia;
  • heavy bleeding;
  • hemorrhagic stroke.

Basically, these complications occur after surgery.

A favorable prognosis is given subject to the clinical recommendations of the treating specialist and the timely intake of necessary medications. But even in these cases, complications often arise. If the rules are not followed, death is not excluded.

The patient should be registered with a therapist and hematologist until the end of his life, since it is not possible to fully recover.

A person can get disability in severe forms of the disease (the content of factor VIII in the blood is below 5%).


Often the disease manifests itself as a congenital pathology of hemostasis, in such cases it cannot be avoided. However, there are preventive measures that must be taken in the presence of von Willebrand disease:

  1. At any visit to the doctor, notify about the presence of pathology.
  2. Do not take anti-inflammatory non-steroidal medicines.
  3. Eliminate blood thinners.
  4. Do not engage in traumatic sports.
  5. Minimize the possibility of injury.
  6. Use iron-fortified foods.
  7. Lead the right way of life.
  8. Control weight

Surgical intervention with such a pathology is allowed to be carried out only in cases where other methods of treatment do not give results.

This pathology is heredity and there is no way to prevent it.

Preventive measures can only prevent severe and frequent bleeding:

  • Before conceiving a child, a consultation with biological parents by a hematologist;
  • Child monitoring (dispensary);
  • Visit your doctor regularly;
  • It is forbidden to take aspirin;
  • Avoid traumatic situations;
  • Healthy living
  • Food culture.

The implementation of these measures will avoid bleeding inside the joints and inside the muscle tissue. Will not allow von Willebrand disease to go into a complicated form.

A patient with von Willebrand disease undergoes treatment in two cases: as a prophylaxis and in case of acute bleeding. Special preparations containing cryoprecipitate and antihemophilic plasma. They contribute to the active development of factor VIII within 36 hours. It is applied once every two days. And it is also successfully practiced for pregnant women before starting labor in order to minimize blood loss. Transfusion therapy is repeated on the sixth day after the birth of the baby.

The types of therapy used against the disease:

  • the use of a hemostatic sponge for skin and mucous bleeding;
  • conducting replacement therapy with the plasma component to increase the PV in the blood;
  • the use of drug therapy to compensate for PV disease.

An anti-disease drug is often prescribed that stimulates an increase in blood of von Willebrand factor. Used as an injection or spray. In case of its ineffectiveness, the drug is canceled and the introduction of replacement therapy is carried out.

A common treatment tactic is the use of replacement therapy – injection of von Willebrand factor.

There is no way to prevent disease to completely eliminate the disease. The disease is transmitted from generation to generation by genetic component. In order to exclude the possibility of frequent bleeding and the possible appearance of pathology in unborn children, it is necessary:

  • Observe the right lifestyle. Maintain a healthy diet and moderate physical activity, if allowed by the attending physician.
  • Eliminate unnecessary surgical interventions (plastic surgery, cosmetic injections). Carry out only under the strict guidance of a specialist, for life-long indications.
  • Minimize the possibility of injury.
  • Observe regularly with your doctor and take blood for analysis.
  • Refusal to take drugs that affect platelet formation (reduce the ability of blood coagulation). The most common is Aspirin.
  • Constant observation in a special hematological clinic.
  • Conducting a genetic examination of spouses planning children using blood sampling for research.

It is also recommended to exclude the possibility of obesity. This process puts a serious strain on the blood vessels. It leads to regular hemorrhages alternating one after another, including into the joint cavity. It is necessary to maintain muscle mass, flexibility. During exercise, exercise, maintain moderation, minimize the possibility of injury, rupture of muscle tissue, eliminate blood flow from the vessels.

Complete prevention of a genetic disease is not possible. If you follow the recommendations of specialists, you can significantly reduce the amount of bleeding.

You should learn how to teach a sick child to independently inject von Willebrand factor into the blood. This ability will help you in a timely manner to help yourself and avoid a lot of bleeding. For any third-party diseases, a mandatory and urgent specialist consultation is indicated. Self-medication of von Willebrand factor is extremely dangerous for the life of a patient with a blood coagulation disease. Many drugs that have not received the approval of a doctor, and can cause great irreversible damage to health and severe bleeding.

Treatment prognosis and complications: when disability is assigned

As a rule, with the first type of disease, complications and consequences for the child are not observed, however, with a severe degree of the disease, severe consequences can occur, expressed in severe bleeding and hemorrhage due to damage to internal organs.

Von Willebrand disease is very dangerous. Even knowing what it is, and following all the recommendations of a doctor, it is not always possible to avoid complications. They are as follows:

  • Chronic anemia.
  • Deterioration of joint performance if hemorrhage was observed in its cavity.
  • Thrombosis that occurs after therapeutic measures.


In healthy people, when a blood vessel is damaged, small blood plates are sent to the site of bleeding, stick together with each other and close the resulting defect. In patients, this process is disrupted, and the blood loses its ability to clot.

A specific symptom of the disease is bleeding of various intensities, vastness and localization. The causes of prolonged bleeding are traumatic injuries, surgical interventions, and dental procedures. In this case, patients develop weakness, dizziness, pallor of the skin, palpitations, the blood pressure decreases, and a pre-syncope condition occurs. The clinical picture of the pathology is largely determined by the magnitude and speed of blood loss.

In children, hemorrhagic diathesis is most severe after acute respiratory infections and other acute infections. During intoxication, vascular permeability increases, which leads to the appearance of spontaneous bleeding. Willebrand’s disease is an incurable pathology with a wave-like course, in which periods of exacerbation are replaced by a complete absence of hemorrhages.

The main manifestations of hemorrhagic syndrome with Willibrand disease:

  1. Bleeding from the digestive tract occurs after taking drugs from the NSAID group and disaggregants. In patients, ulcers of the gastrointestinal tract and hemorrhoids usually bleed. Arteriovenous anastomoses often cause recurring bleeding. Symptoms of gastric bleeding are melena – a tarry black loose stool and vomiting of altered dark blood.
  2. Hemarthrosis – hemorrhage in the joint cavity, manifested by pain, limited function, swelling and redness of the skin, increased pain on palpation. The joint increases in volume, becomes spherical, its contours are smoothed out. With continued bleeding in the joint, the skin becomes cyanotic, soft tissues – tight, tense, local hyperthermia appears.
  3. In especially severe cases, hemorrhagic syndrome in patients is combined with signs of mesenchymal dysplasia. Local vascular and stromal dysplasias provoke persistently repeated bleeding mainly of one localization.

Symptoms of a mild form of pathology:

  • Frequent nosebleeds
  • Heavy menstrual bleeding,
  • Prolonged bleeding with minor skin damage,
  • Petechiae,
  • Hemorrhages after injuries.

Clinical signs of a severe form:

  • Blood in the urine is accompanied by lower back pain and dysuria symptoms,
  • Extensive hematomas after a slight bruise compress large vessels and nerve trunks, which is manifested by pain,
  • Hemarthrosis, accompanied by pain in the affected joint, its edema, local hyperthermia,
  • Prolonged bleeding from the gums after brushing your teeth,
  • Bleeding from the pharynx and nasopharynx can lead to bronchial obstruction,
  • Hemorrhages in the lining of the brain lead to damage to the central nervous system or death.

In this case, the symptoms of the disease are almost identical to hemophilia.

Indications for analysis

The study is prescribed for patients with atherosclerosis.

Indications for analysis can be both symptoms that do not exclude pathology from the coagulation function, and existing diseases leading to damage to the vascular wall. In the latter case, von Willebrand factor allows you to indirectly assess how much endothelium (the inner part of the vessels) is involved in the pathological process and is affected by the disease. The definition of this indicator is shown in a number of cardiovascular diseases:

  • arterial hypertension
  • conditions after myocardial infarction,
  • coronary heart disease
  • arteriosclerosis of arteries.

Increased values ​​will indicate the progression of vascular and heart diseases, a decrease in the level will let you know about the effectiveness of the treatment.

Gum Bleeding – Indication for Analysis

However, the analysis is primarily indicated for suspected von Willebrand disease. The main symptoms include:

  • Menorrhagia – prolonged and excessively profuse menstruation up to uterine bleeding;
  • Nosebleeds that are difficult to stop;
  • Prolonged and large blood loss with relatively minor damage to the skin, mucous membranes;
  • Hemarthrosis – damage to the joints with profuse hemorrhage in the joint cavity, which occurs after minor bruises;
  • Bleeding from the gums when brushing your teeth;
  • Mild hematomas (bruises), often not associated with injuries.

The analysis is recommended when planning a pregnancy for both future parents if they themselves or direct relatives have symptoms of a clotting pathology.


With a mild form of the disease, treatment is carried out if necessary, for example, due to an emergency operation, with injuries or with a view to prevention.

For the treatment of children, the means are used:

  • cryoprecipitate;
  • freshly frozen plasma ;.
  • homeopathic remedies.

The constant intake of medications is necessary for people with a severe form of pathology. To do this, prescribe drugs that have a hemostatic effect and containing the component necessary for coagulation.

Women are also recommended additional hormonal treatment.

Sometimes, in rare cases, a surgical operation is performed to ligate the vessels or remove some part to stop the hemorrhage.

With a pathology that proceeds in a severe stage, the patient is sometimes assigned a disability. But more often people with a plasma element content below 5% do not live up to thirty years.

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Tatyana Jakowenko

Editor-in-chief of the Detonic online magazine, cardiologist Yakovenko-Plahotnaya Tatyana. Author of more than 950 scientific articles, including in foreign medical journals. He has been working as a cardiologist in a clinical hospital for over 12 years. He owns modern methods of diagnosis and treatment of cardiovascular diseases and implements them in his professional activities. For example, it uses methods of resuscitation of the heart, decoding of ECG, functional tests, cyclic ergometry and knows echocardiography very well.

For 10 years, she has been an active participant in numerous medical symposia and workshops for doctors - families, therapists and cardiologists. He has many publications on a healthy lifestyle, diagnosis and treatment of heart and vascular diseases.

He regularly monitors new publications of European and American cardiology journals, writes scientific articles, prepares reports at scientific conferences and participates in European cardiology congresses.