Von Willebrand disease is an autosomal incompletely dominant genetic disorder. Due to deficiency or abnormality of von Willebrand factor (vWF), its clinical manifestations include bleeding and prolonged bleeding time.

bubble_chart Diagnosis

1. Medical History and Symptoms

(1) History Inquiry: Pay attention to the age of onset, predisposing factors for bleeding, severity and location of bleeding, and whether the severity and frequency of bleeding tend to decrease with age. For females, inquire about a history of hypermenorrhea or excessive postpartum metrorrhagia.

(2) Clinical Symptoms: Epistaxis, gum bleeding, and skin ecchymosis are common, while hematuria, gastrointestinal bleeding, and intracranial bleeding are occasional. Joint bleeding is rare. Severe bleeding occurs after trauma or surgery, and hemostasis is difficult. Females may experience hypermenorrhea.

2. Physical Examination Findings

Ecchymosis may be observed on the skin and mucous membranes, with oozing at wound sites. If joint bleeding occurs, joint swelling, tenderness, and restricted movement may be present.

3. Auxiliary Examinations

1. Blood Test: Normal counts and morphology of red blood cells, white blood cells, and platelets.

2. Prolonged bleeding time or positive aspirin tolerance test.

3. Activated partial thromboplastin time (APTT) prolonged or normal.

4. Reduced or normal platelet adhesion rate (64.2%±8.3%).

5. Reduced or normal factor VIII coagulation activity.

vWF antigen (vWF:Ag) decreased or normal.

Positive vascular fragility test.

4. Differential Diagnosis

Should be differentiated from coagulation factor deficiency disorders such as thrombasthenia, giant platelet syndrome, and hemophilia.

1. Avoid drugs that affect platelet function: aspirin, dipyridamole, indomethacin, etc.

2. Replacement therapy: Infusion of cryoprecipitate. Usage and dose refer to the treatment of hemophilia A.

3. 6-Aminocaproic acid: 75mg/kg, orally, every 6h, for 7～10d. Or 4～6g, intravenous drip. <30min，3～4次/d。

4. DDAVP (1-deamino-8-D-arginine vasopressin): 0.3mg/kg, dissolved in 50ml saline, intravenous injection >15min, 3～4 times/d. Contraindicated in type II B von Willebrand disease.

5. Local bleeding: Mainly hemostasis by compression.