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Home > West Medicine > Internal Medicine > Hematology
D68.000x001

von Willebrand Disease

血液科

Common symptoms

创伤、出血倾向、颅内出血、齿龈出血、产后出血、鼻衄、胃肠道出血

The pathology of disease

von Willebrand disease (vWD) is the most common hereditary bleeding disorder, caused by quantitative or qualitative deficiency of von Willebrand factor (vWF), which impairs platelet adhesion to damaged vessel walls and serves as carrier for factor VIII. Clinical: mucocutaneous bleeding (epistaxis, menorrhagia, gingival bleeding), prolonged bleeding time; autosomal dominant (types 1/2) or recessive (type 3).

Common Symptoms

Mucocutaneous bleeding; epistaxis; menorrhagia; gingival bleeding; easy bruising; prolonged bleeding after surgery

Causes and Pathogenesis

Mutations in the vWF gene cause quantitative (type 1/3) or qualitative (type 2) vWF deficiency, impairing primary hemostasis (platelet adhesion) and reducing factor-VIII half-life.

Treatment

Desmopressin (DDAVP) for type 1; vWF/Factor-VIII concentrate for type 2/3; antifibrinolytics; estrogen for menorrhagia

Preventive Measures

Avoid aspirin/NSAIDs; dental/surgical prophylaxis; genetic counseling.

Department to Visit

It is recommended to visit the Hematology department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.