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Symptoms of Willebrand's Disease
Last reviewed: 20.11.2021
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The main symptom of von Willebrand disease is increased bleeding during trauma or pathological processes. Since Willebrand's disease suffers from a bleeding stop function, this disease is characterized by primary bleeding that begins immediately after the injury.
The nature and severity of hemorrhagic syndrome in Willebrand disease depend on the form of the disease. In general, we can conditionally distinguish three options.
- Hemorrhagic syndrome by microcirculatory type. Characteristic for 1, 2A, 2B, 2M types of von Willebrand disease. Typical skin hemisyndrom in the form of ecchymoses, petechia, bleeding from injured mucous membranes, prolonged bleeding from the holes of the removed or dropped teeth, nasal bleeding, uterine bleeding in girls after the onset of menstruation, intraoperative and postoperative hemorrhages, gastrointestinal bleeding and bleeding from the urinary tract. Less common bleeding from injection sites and hematoma of soft tissues after various injuries.
- Clinically reminiscent of hemophilia. Patients expressed hemorrhagic syndrome for mixed (hematoma and microcirculatory) type. It is characteristic for patients with diseases of Willebrand type 3, less often for patients with severe forms of other types. The first manifestations of the disease occur already in the period of the newborn: dermal hemisyndrome, hematoma and bleeding from the injection sites. Later - soft tissue hematomas; bleeding with injuries of the mucous membranes of the mouth, with a change of teeth; bleeding from skin and mucous membrane wounds, nasal, intestinal and urinary bleeding. After the onset of menstruation, uterine bleeding is common in girls. Hemorrhages in the joints, as well as in hemophilia, may appear in the first year of life. They are characterized by intraoperative and postoperative hemorrhage.
- The clinical picture is similar to that observed in patients with hemophilia A with a comparable level of coagulation factor VIII: hematoma type of hemorrhage, rarely accompanied by joint damage. Characterized by cutaneous gemsindrom in the form of hematomas, delayed, occurring after a few hours or days after the onset of trauma, bleeding during injuries and after operations. In patients with Willebrand type 2M diseases, posttraumatic hematomas of soft tissues may occur.
Complications of hemorrhagic manifestations
Children with Willebrand disease may develop chronic post-hemorrhagic anemia. Arthropathy occurs in children with recurrent hemarthrosis in the third type of von Willebrand disease. Single cases of formation of pseudotumors are described.
In addition to hemorrhagic manifestations, often Willebrand's disease is combined with the phenomena of mesenchymal dysplasia, which can affect the severity of hemorrhagic syndrome.