Management

• Avoid aspirin and NSAIDs.

• Mild bleeding symptoms—easy bruising, bleeding from cuts may settle with local pressure.

• Tranexamic acid (TXA) is a useful antifibrinolytic drug (15mg/kg PO tds).

• TXA mouthwash 5% is useful for dental work.

• Moderate disease and minor surgery

- DDAVP (0.3mg/kg SC or by slow IV injection/infusion). Fewer side effects with SC route.

- Most responders have type 1 vWD but may work in some type 2 patients. Avoid in type 2B (may reduce platelets).

• Major surgery, bleeding symptoms or severe disease.

- If DDAVP insufficient use vWF rich factor VIII concentrate e.g. intermediate purity VIII concentrate, e.g. Alphanate, BPL 8Y, Haemate P.

- Monitor treatment with VWF:Ricof or VWF:Ag. Bleeding time or PFA-100 (see below) may not correct despite good clinical response. Treat post-op for 7-10 days.

• Pregnancy—VIII and VWF rise in pregnancy so rarely presents a problem for type 1. Post-partum vWF falls so watch out for PPH in mod/severely affected women. Give DDAVP or vWF concentrate to maintain levels >30% if clinical problem. In Type 2B abnormal HMW multimers can cause platelet aggregation and thrombocytopenia in pregnancy. Avoid TXA in pregnancy/type 2B as there may be risk of thrombosis.

• Menorrhagia—may be major problem. TXA for first 3 days of the menstrual period helps some patients. Combined oral contraceptive pill is useful. Mirena (hormone impregnated) coil very effective in some patients.

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