Management of Von Willebrand Disease with Normal INR
For a patient with von Willebrand disease (VWD) and a normal INR of 1.2, referral to hematology is appropriate, and treatment should be based on VWD type and bleeding risk, with desmopressin as first-line therapy for most patients with type 1 and 2A VWD.
VWD Classification and Initial Assessment
- VWD is the most common inherited bleeding disorder, caused by quantitative or qualitative defects in von Willebrand factor (VWF) 1
- A normal INR (1.2) is expected in VWD as INR primarily measures the extrinsic coagulation pathway, not VWF function 2
- Accurate diagnosis of VWD subtype is essential as treatment varies by type 3
Treatment Approach Based on VWD Type
For Type 1 and Type 2A VWD:
- Desmopressin (0.3 μg/kg) is the first-line treatment for patients with mild to moderate type 1 and 2A VWD 2, 4
- Desmopressin works by releasing stored VWF from endothelial cells, increasing plasma levels of both VWF and factor VIII 4
- Administration should be 30 minutes prior to any invasive procedure to maintain hemostasis 4
For Type 2B, 2M, 2N, and Type 3 VWD:
- VWF/FVIII concentrate is the preferred treatment option 2
- If VWF/FVIII concentrate is unavailable, cryoprecipitate is indicated 2
- Desmopressin is not recommended for type 2B as it may cause thrombocytopenia 2
Monitoring Treatment Response
- Monitor bleeding time, factor VIII coagulant activity, ristocetin cofactor activity, and VWF antigen levels during desmopressin administration 4
- Ensure adequate levels are being achieved to prevent bleeding complications 4
- Bleeding risk is higher when VWF ristocetin cofactor and factor VIII levels are <50 IU/dL 3
Perioperative and Bleeding Management
- For surgical procedures or active bleeding, treatment should be initiated when VWF and factor VIII levels are <50 IU/dL 3
- If no response to desmopressin or if unavailable, specific VWF/FVIII concentrate should be administered 2
- For severe bleeding episodes or major surgery in patients with type 3 VWD, VWF/FVIII concentrate is required 5
Special Considerations
- Test for von Willebrand activity in patients with a history of bleeding diathesis 2
- Patients on anticoagulants or antiplatelet medications have increased bleeding risk and require careful management 2
- Prophylactic treatment may be considered for patients with severe forms of VWD who experience recurrent bleeding episodes 5
- Antifibrinolytic therapy may be used as adjunctive treatment, especially for mucosal bleeding 6
Long-term Management
- Regular follow-up with hematology is essential to monitor disease status and adjust treatment 7
- Prophylaxis has shown significant reduction in bleeding rates for patients with severe VWD, particularly for epistaxis, joint bleeding, and GI bleeding 5
- Quality of life assessment should be part of routine care as VWD can have significant physical and mental health impacts 6
Potential Pitfalls and Caveats
- Desmopressin is ineffective for patients with severe type 3 VWD and factor VIII levels <5% 4
- Tachyphylaxis can occur with repeated desmopressin doses given within 24 hours 2
- Delayed bleeding may occur despite adequate initial prophylaxis, requiring continued monitoring and potential extended treatment 3
- FFP is not indicated if PT/INR and aPTT are normal, as is the case with this patient 2