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Von Willebrand disease (VWD) is the most common inherited coagulation disorder (acquired forms have also been described). The condition is caused either by a reduced level of von Willebrand factor (vWF) (quantitative defect) or by abnormal function of the protein (qualitative defect).
Von Willebrand factor is a multimeric protein that mediates platelet adhesion by forming a bridge between platelets and the damaged vascular wall. It also binds clotting factor VIII, thereby regulating its plasma level.
The clinical manifestations vary depending on the plasma concentration of von Willebrand factor (vWF). Diagnosis is based on medical history and laboratory investigations, including quantitative vWF measurement, ristocetin cofactor activity (vWF: RCo), and factor VIII levels.
Statistics
VWD is the most common inherited hemorrhagic disorder, affecting about 1% of the general population. It occurs with equal frequency in males and females; however, symptomatic disease is more frequently seen in women due to menstrual bleeding.
There are three main hereditary types of VWD: type 1 accounts for 60–80% of cases, type 2 accounts for 20–30%, and type 3 accounts for approximately 5% of cases.
The true prevalence of acquired VWD is unknown. It has been described in association with malignancies, as well as in up to 20% of patients with prosthetic heart valves.
Clinical Manifestations
Clinical features vary depending on the type of disease. Type 1 is usually mild or asymptomatic, whereas types 2 and 3 present with progressively more severe manifestations. Although most patients are asymptomatic, when symptoms occur, they may include:
Mucocutaneous bleeding: gingival bleeding, epistaxis, petechiae, recurrent and easy bruising, menorrhagia,
Excessive bleeding after dental or surgical procedures,
Gastrointestinal bleeding (often associated with arteriovenous malformations),
Postpartum hemorrhage,
Hemarthroses, observed primarily in types 2N and 3 (joint cavity bleeding with pain, swelling, and restricted range of motion),
Hematomas,
In children: umbilical cord bleeding, cephalohematoma, prolonged bleeding after vaccinations, and bleeding following circumcision.
