Table 4.

Clinical scenarios encountered in the management of patients with low VWF

Alternate clinical scenariosClinical assessmentSuggested management strategy
Case 1 22-y-old male with low VWF Assess personal and family bleeding history Single dose of DDAVP before DE 
Baseline plasma VWF:Ag, 35 IU/dL; VWF:RCo, 32 IU/dL Calculate ISTH BAT score If no previous record of DDAVP response, assess plasma VWF levels at baseline and 1, 2, and 4 h post-DDAVP 
Requires a surgical molar DE Determine whether the patient has previously been treated for any procedures with tranexamic acid and/or DDAVP Tranexamic acid, 1 g, 3 times a day for 3-5 d postprocedure 
  Contact details in case of bleeding 
Case 2 65-y-old with history of low VWF levels Assess personal and family bleeding history Treatment plan will be based upon global risk assessment for bleeding and thrombotic potential 
Baseline plasma VWF:Ag, 32 IU/dL; VWF:RCo, 30 IU/dL Calculate ISTH BAT score If risk of stroke outweighs bleeding risk, consider introduction of anticoagulation with regular ongoing follow-up at 3 monthly intervals to reassess 
More recent plasma VWF:Ag and VWF:RCo levels now consistently >50 IU/dL Consider any comorbidities/medications that may contribute to current bleeding risk Provide contact details in case of bleeding 
Has developed persistent atrial fibrillation Determine CHA2DS2-VASc score to assess risk of CVA  
Case 3 70-y-old woman with low VWF levels Assess personal and family bleeding history If elevated bleeding history, treat with tranexamic acid cover (1 g preoperatively and 1 g, 3 times a day postoperatively for 48-72 h 
Baseline plasma VWF:Ag, 40 IU/dL; VWF:RCO, 44 IU/dL Calculate ISTH BAT score Daily review by Coagulation Service to determine when tranexamic acid can be discontinued and LMWH introduced. Thromboembolic Deterrent Stockings. 
More recent plasma VWF levels consistently >70 IU/dL Consider any comorbidities/medications that may contribute to current bleeding or thrombotic risks Early mobilization as surgically appropriate 
Requires elective total knee replacement   
Alternate clinical scenariosClinical assessmentSuggested management strategy
Case 1 22-y-old male with low VWF Assess personal and family bleeding history Single dose of DDAVP before DE 
Baseline plasma VWF:Ag, 35 IU/dL; VWF:RCo, 32 IU/dL Calculate ISTH BAT score If no previous record of DDAVP response, assess plasma VWF levels at baseline and 1, 2, and 4 h post-DDAVP 
Requires a surgical molar DE Determine whether the patient has previously been treated for any procedures with tranexamic acid and/or DDAVP Tranexamic acid, 1 g, 3 times a day for 3-5 d postprocedure 
  Contact details in case of bleeding 
Case 2 65-y-old with history of low VWF levels Assess personal and family bleeding history Treatment plan will be based upon global risk assessment for bleeding and thrombotic potential 
Baseline plasma VWF:Ag, 32 IU/dL; VWF:RCo, 30 IU/dL Calculate ISTH BAT score If risk of stroke outweighs bleeding risk, consider introduction of anticoagulation with regular ongoing follow-up at 3 monthly intervals to reassess 
More recent plasma VWF:Ag and VWF:RCo levels now consistently >50 IU/dL Consider any comorbidities/medications that may contribute to current bleeding risk Provide contact details in case of bleeding 
Has developed persistent atrial fibrillation Determine CHA2DS2-VASc score to assess risk of CVA  
Case 3 70-y-old woman with low VWF levels Assess personal and family bleeding history If elevated bleeding history, treat with tranexamic acid cover (1 g preoperatively and 1 g, 3 times a day postoperatively for 48-72 h 
Baseline plasma VWF:Ag, 40 IU/dL; VWF:RCO, 44 IU/dL Calculate ISTH BAT score Daily review by Coagulation Service to determine when tranexamic acid can be discontinued and LMWH introduced. Thromboembolic Deterrent Stockings. 
More recent plasma VWF levels consistently >70 IU/dL Consider any comorbidities/medications that may contribute to current bleeding or thrombotic risks Early mobilization as surgically appropriate 
Requires elective total knee replacement   

CVA, cerebrovascular accident; LMWH, low molecular weight heparin.

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