Table 2.

Suggested VWD management strategies

Type of bleedingTarget VWF:RCo / FVIII level (IU/dL)DDAVP responsiveDDAVP nonresponsiveAdjunctive therapies
Acute bleeding     
Minor >30 IU/dL TA ± DDAVP TA ± VWF concentrate  
Major (life threatening) ≈100 IU/dL TA + VWF concentrate TA + VWF concentrate Consider platelets if bleeding persists 
Surgery     
Minor >30 IU/dL until healing complete Pre-op: TA; IV DDAVP Pre op: TA + VWF concentrate  
Post-op: TA for 3-5 d Post-op: TA for 3-5 d 
Major ≈100 IU/dL until 36 h post-op Pre-op: TA; IV DDAVP Pre-op: TA + VWF  
Maintain >50 IU/dL for 5-10 d May require VWF concentrate if tachyphylaxis concentrate 
Dental extraction >50 IU/dL for 12 h Pre: DDAVP Pre: VWF concentrate  
Post: TA for 3 d Post: TA for 3 d 
Menorrhagia  TA to cover days of heaviest flow TA to cover days of heaviest flow OCP 
Monthly intranasal DDAVP Levonorgestrel intrauterine device (Mirena) 
Iron supplementation 
Endometrial ablation 
Hysterectomy 
Type of bleedingTarget VWF:RCo / FVIII level (IU/dL)DDAVP responsiveDDAVP nonresponsiveAdjunctive therapies
Acute bleeding     
Minor >30 IU/dL TA ± DDAVP TA ± VWF concentrate  
Major (life threatening) ≈100 IU/dL TA + VWF concentrate TA + VWF concentrate Consider platelets if bleeding persists 
Surgery     
Minor >30 IU/dL until healing complete Pre-op: TA; IV DDAVP Pre op: TA + VWF concentrate  
Post-op: TA for 3-5 d Post-op: TA for 3-5 d 
Major ≈100 IU/dL until 36 h post-op Pre-op: TA; IV DDAVP Pre-op: TA + VWF  
Maintain >50 IU/dL for 5-10 d May require VWF concentrate if tachyphylaxis concentrate 
Dental extraction >50 IU/dL for 12 h Pre: DDAVP Pre: VWF concentrate  
Post: TA for 3 d Post: TA for 3 d 
Menorrhagia  TA to cover days of heaviest flow TA to cover days of heaviest flow OCP 
Monthly intranasal DDAVP Levonorgestrel intrauterine device (Mirena) 
Iron supplementation 
Endometrial ablation 
Hysterectomy 

OCP, oral contraceptive pill; TA, tranexamic acid.

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