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Symptoms of Willebrand's disease
Last reviewed: 04.07.2025
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The main symptom of von Willebrand disease is increased bleeding during trauma or pathological processes. Since von Willebrand disease affects the function of stopping bleeding, this disease is characterized by primary bleeding that begins immediately after the injury.
The nature and severity of hemorrhagic syndrome in von Willebrand disease depend on the form of the disease. In general, three variants can be conditionally distinguished.
- Hemorrhagic syndrome of the microcirculatory type. Characteristic for types 1, 2A, 2B, 2M of von Willebrand disease. Typical are cutaneous hemisyndrome in the form of ecchymosis, petechiae, bleeding from injured mucous membranes, prolonged bleeding from the sockets of extracted or fallen teeth, nosebleeds, uterine bleeding in girls after the onset of menstruation, intraoperative and postoperative bleeding, gastrointestinal bleeding and bleeding from the urinary tract. Less typical are bleeding from injection sites and soft tissue hematomas after various injuries.
- Clinically resembles hemophilia. Patients have a pronounced hemorrhagic syndrome of mixed (hematoma and microcirculatory) type. It is typical for patients with von Willebrand disease type 3, less often - for patients with severe forms of other types. The first manifestations of the disease occur in the neonatal period: cutaneous hemisyndrome, hematomas and bleeding from injection sites. Later - hematomas of soft tissues; bleeding from injuries to the mucous membranes of the mouth, when changing teeth; bleeding from wounds of the skin and mucous membranes, nosebleeds, intestinal and urinary tract bleeding. After the onset of menstruation, girls often have uterine bleeding. Hemorrhages into the joints, as well as in hemophilia, can appear in the first year of life. They are characterized by intraoperative and postoperative bleeding.
- The clinical picture is similar to that observed in patients with hemophilia A with a comparable level of coagulation factor VIII: hematoma type of bleeding, rarely accompanied by joint damage. Cutaneous hematoma syndrome is characteristic in the form of hematomas, delayed, occurring several hours or days after the onset of injury, bleeding during injuries and after operations. In patients with von Willebrand disease type 2M, post-traumatic hematomas of soft tissues may occur.
Complications of hemorrhagic manifestations
Children with von Willebrand disease may develop chronic posthemorrhagic anemia. Arthropathies occur in children with recurrent hemarthrosis in type 3 von Willebrand disease. Isolated cases of pseudotumor formation have been described.
In addition to hemorrhagic manifestations, von Willebrand disease is often combined with mesenchymal dysplasia, which can affect the severity of hemorrhagic syndrome.