Types of disease
There are three main types. The first, the most common and usually the mildest, means that there is simply less VWF than needed, but it is of normal quality. The second type combines options in which there may be enough protein, but it does not work correctly; Within the type there are several subspecies, and the choice of drug depends on them. The third type is rare: the factor is practically absent, which is why the level of factor VIII also drops, and bleeding resembles hemophilia, including hemorrhages in the joints. There is also an acquired variant associated with other diseases.
- Type 1 - quantitative deficiency, mild course
- Type 2 - qualitative protein defect, several subtypes
- Type 3 - almost complete absence of the factor, severe course
- Acquired syndrome in heart and blood diseases
- The choice of treatment depends on the type
Symptoms
Bleeding in this disease is mainly superficial, from small vessels of the skin and mucous membranes. People report that bruises appear from light blows, and cuts take longer to bleed than usual. Nosebleeds often recur since childhood and are difficult to stop. In women, the leading symptom is heavy and prolonged menstruation, often leading to iron deficiency anemia; increased risk of bleeding after childbirth. A separate group of manifestations is unexpectedly severe bleeding after dental and surgical interventions, which sometimes lead to a diagnosis.
- Easy bruising
- Recurrent nosebleeds
- Bleeding gums when brushing teeth
- Heavy and prolonged menstruation
- Prolonged bleeding from cuts
- Bleeding after tooth extraction and surgery
- Iron deficiency anemia as a consequence
Diagnostics
The examination begins with detailed questioning about bleeding from the patient himself and from relatives; There are special questionnaires for this purpose. A standard coagulogram may be normal, so it alone is not enough: the aPTT does not always lengthen. Key tests are the amount of von Willebrand factor, its activity and the level of factor VIII. The results depend on the blood type, stress, inflammation, pregnancy and contraceptive use, so if the values are borderline, the tests are repeated. If a deficiency is confirmed, clarifying studies are carried out to determine the type and select treatment.
- Assess patient and family bleeding history
- Von Willebrand factor: quantity and activity
- Clotting factor VIII
- APTT and basic coagulogram
- Complete blood count and ferritin
- Repeated testing for borderline results
- Clarifying tests to determine type
Treatment and preparation for interventions
Permanent treatment is usually not required: therapy is prescribed for bleeding and before procedures. For mild types, a drug is used that releases von Willebrand factor reserves from the vascular wall; its effectiveness is checked in advance by a breakdown. For severe types and major operations, concentrates containing von Willebrand factor and factor VIII are administered. Antifibrinolytics are widely used, especially for bleeding from the mucous membranes and in dentistry, as well as local agents. Women with heavy menstruation are helped by hormonal therapy, selected by a gynecologist together with a hematologist.
- Drugs that release intrinsic factor
- Von Willebrand factor and factor VIII concentrates
- Antifibrinolytics for bleeding from mucous membranes
- Local hemostatic agents
- Hormonal therapy for heavy menstruation
- Iron supplements for confirmed anemia
- Mandatory planning of any operation with a hematologist
Living with illness
You can live a full life with this disease if you follow a few rules. It is important to avoid drugs that affect platelets without consulting your doctor: acetylsalicylic acid and a number of anti-inflammatory drugs increase bleeding. Before any operation, including tooth extraction and childbirth, it is necessary to notify doctors in advance and coordinate preparations. It is useful to have a document with you with the diagnosis and type of disease. Sports are not prohibited, but it is better to replace contact martial arts with less traumatic types. Pregnancy is managed jointly by a gynecologist and a hematologist.
- Warn the dentist and surgeon about the diagnosis in advance
- Do not take acetylsalicylic acid unless prescribed
- Caution with anti-inflammatory drugs
- Carry with you a certificate with the diagnosis and type
- Choose non-contact sports
- Pregnancy should be discussed with a hematologist
- Monitor hemoglobin and ferritin levels