Maladie de von Willebrand
Von Willebrand's disease is a congenital disease that bleeds easily. In affected patients, a blood-clotting protein called von Willebrand factor is only slightly or not functioning normally. Von Willebrand's disease is classified as an incurable disease. But with the right treatment, patients can lead normal lives.
Symptoms of von Willebrand's disease
Symptoms of von Willebrand's disease can be mild or even not appear at all. The patient is not aware of it. The severity of the disease is different for each patient.
The symptoms of von Willebrand's disease are as follows:
Symptoms of von Willebrand's disease will be more easily seen in women, especially during menstruation, including:
Causes of von Willebrand's disease
Von Willebrand's disease is caused by mutations in the VWF gene, a gene that gives the body instructions to produce von Willebrand factor. Mutations in the VWF gene can reduce the amount of von Willebrand factor or interfere with the function of von Willebrand factor.
Von Willebrand factor itself functions to form blood clots by attaching platelets to the walls of injured or bleeding tissue to form blood clots. Formation of blood clots is important to stop bleeding. However, in patients with this disease, there are only a few von Willebrand factors in the body, or there is a disruption in the function of these factors. This condition makes the platelets can not be attached, resulting in blood clots and uncontrolled bleeding.
This genetic disorder is usually inherited from parents. However, this genetic anomaly can occur alone when a person is an adult, even though his parents do not have the same disease.
Diagnosis of von Willebrand's disease
During the initial examination, the doctor will ask you about the patient's and family's illness history. Then, the doctor will do a blood test, such as:
Remember that test results can vary because they are influenced by a number of factors such as infection, stress, pregnancy, or drug use. So the patient can be asked to repeat the blood test.
Treatment of von Willebrand's disease
Although von Willebrand's disease cannot be cured, treatment can help prevent and stop bleeding. The treatment method chosen depends on the patient's condition, type, severity of the disease and the patient's response to previous treatment.
Some types of treatments for Von Willebrand's disease are:
Complications of von Willebrand's disease
Symptoms of von Willebrand's disease can be mild or even not appear at all. The patient is not aware of it. The severity of the disease is different for each patient.
The symptoms of von Willebrand's disease are as follows:
- Severe bleeding after tooth extraction or surgery
- Nosebleeds that don't stop in 10 minutes
- There is blood in the urine or stool
- Bruised skin
Symptoms of von Willebrand's disease will be more easily seen in women, especially during menstruation, including:
- Menstruation lasts a long time or a lot of blood comes out.
- It takes two electrodes at a time to limit the rate of menstrual blood.
- Change towels or tampons more than once an hour.
- Symptoms of anemia appear, such as fatigue, weakness or shortness of breath.
Causes of von Willebrand's disease
Von Willebrand's disease is caused by mutations in the VWF gene, a gene that gives the body instructions to produce von Willebrand factor. Mutations in the VWF gene can reduce the amount of von Willebrand factor or interfere with the function of von Willebrand factor.
Von Willebrand factor itself functions to form blood clots by attaching platelets to the walls of injured or bleeding tissue to form blood clots. Formation of blood clots is important to stop bleeding. However, in patients with this disease, there are only a few von Willebrand factors in the body, or there is a disruption in the function of these factors. This condition makes the platelets can not be attached, resulting in blood clots and uncontrolled bleeding.
This genetic disorder is usually inherited from parents. However, this genetic anomaly can occur alone when a person is an adult, even though his parents do not have the same disease.
Diagnosis of von Willebrand's disease
During the initial examination, the doctor will ask you about the patient's and family's illness history. Then, the doctor will do a blood test, such as:
- Von Willebrand factor antigen. With this test, the doctor can determine the level of von Willebrand factor in the blood by measuring the level of a number of proteins in the blood.
- Ristocetin cofactor activity. In this test, the doctor will measure the effectiveness of the von Willebrand factor in the process of blood clotting.
Remember that test results can vary because they are influenced by a number of factors such as infection, stress, pregnancy, or drug use. So the patient can be asked to repeat the blood test.
Treatment of von Willebrand's disease
Although von Willebrand's disease cannot be cured, treatment can help prevent and stop bleeding. The treatment method chosen depends on the patient's condition, type, severity of the disease and the patient's response to previous treatment.
Some types of treatments for Von Willebrand's disease are:
- Tranexamic Acid. This drug is used to slow down the process of blood clots that have formed, so the bleeding can stop.
- Birth control pills. In patients, contraceptive pills can be used to control heavy bleeding during menstruation. The hormone estrogen in birth control pills can also increase von Willebrand factor production.
- Desmopressin. Desmopressin is a synthetic hormone that stimulates the body to release more von Willebrand factors that are stored in blood vessel walls.
Complications of von Willebrand's disease
- If they are not treated properly, people with von Willebrand's disease may experience complications such as:
- Pain and swelling This condition occurs when abnormal bleeding occurs in soft tissue or joints.
- Anemia Excessive menstrual bleeding can cause iron deficiency anemia.
- Von Willebrand's disease can cause uncontrolled bleeding to death. Therefore, medical treatment must be given immediately if there is serious bleeding.
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