Diagnosis and Management of Von Willebrand Disease

Clinical Evaluation and Diagnosis

The National Heart, Lung, and Blood Institute (NHLBI) guidelines identify persons presenting with personal and/or family history of bleeding symptoms as critical scenarios where VWD evaluation is warranted, applicable to patients with bleeding symptoms and NSAID use 1
The NHLBI guidelines also recommend VWD evaluation for asymptomatic persons undergoing surgical/invasive procedures who need bleeding risk assessment 1
The American Society of Hematology recommends asking preoperative screening questions, including family history of bleeding problems, to assess the risk of VWD 3
Patients with mucocutaneous bleeding history should undergo initial VWD assays, including VWF antigen, VWF ristocetin cofactor activity, and Factor VIII coagulant activity, to establish diagnosis, type, and severity 1, 2

Initial hemostasis screening tests, including complete blood count (CBC) with platelets and peripheral smear, prothrombin time (PT), and activated partial thromboplastin time (aPTT), are recommended, but PT and aPTT alone will miss VWD and mild platelet function disorders 1, 3
The American Society of Hematology warns that PT and aPTT alone will miss VWD and mild platelet function disorders, and recommends additional testing, including VWF assays 5
A ratio of VWF:RCo to VWF:Ag below 0.5-0.7 is an interpretation trigger for further testing 3

Acquired von Willebrand Syndrome (AVWS) should be considered if the patient has abnormal VWF results and bleeding symptoms without a personal/family history consistent with hereditary VWD 2
The American College of Hematology recommends against relying solely on PT/aPTT to exclude bleeding disorders, as this misses VWD entirely 5
A structured bleeding history using validated assessment tools is essential for accurate diagnosis 5