von Willebrand disease

Common inherited bleeding disorder due to quantitative or qualitative defects of von Willebrand factor leading to impaired platelet adhesion and secondary reduction of factor VIII levels; presentation is mucocutaneous bleeding.

• Most prevalent inherited bleeding disorder; usually mild and responsive to desmopressin, but peri-procedural planning is crucial.

• Easy bruising, epistaxis, menorrhagia, and bleeding after dental work.
• Labs: normal platelet count with prolonged bleeding time; PT normal; PTT normal or mildly prolonged.

1. Ristocetin aggregation test is impaired.
2. Type 1 disease responds to desmopressin (endothelial release of vWF and factor VIII).
3. Severe disease or major surgery: vWF-containing concentrates plus or minus tranexamic acid.
4. Avoid aspirin and NSAIDs which worsen mucosal bleeding.

1. Type 1: desmopressin plus or minus tranexamic acid for procedures.
2. Type 2/3 or major surgery/trauma: vWF and factor VIII concentrate.
3. Menorrhagia: combined hormonal contraception or levonorgestrel IUD; add tranexamic acid during menses if needed.

• Platelet adhesion uses vWF with GPIb; platelet-to-platelet aggregation uses GPIIb/IIIa.
• Pregnancy and stress can raise vWF levels; bleeding may improve transiently.

• Massive bleeding unresponsive to desmopressin: give vWF concentrate.
• Avoid platelet-inhibiting drugs such as aspirin or NSAIDs.

1. Mucocutaneous bleeding history; order CBC with platelets, PT, PTT, and ristocetin assay.
2. If consistent with vWD, treat per severity (desmopressin vs vWF concentrate).
3. For procedures: test desmopressin response beforehand; add antifibrinolytic for oral or ENT surgery.

• Teen with heavy menses and epistaxis, normal platelets, prolonged bleeding time, and mild PTT elevation.
• Dental extraction in mild vWD; best prophylaxis is desmopressin.