Von Willebrand Disease
You are informed that there is a history of von Willebrand disease in her family and she shows you a registration card from when she was 6 years old on which a VWF antigen level of 0.42 iu/ml is documented.
Von Willebrand Disease (vWD)
- Von Willebrand disease is the most common inherited bleeding disorder in the general population. It results from deficiency or dysfunction of vWF.
- Why both bleeding time and aPTT are prolonged:
- Deficient vWF impairs platelet adhesion to collagen → prolonged bleeding time
- Deficient vWF reduces Factor VIII levels (because vWF normally carries and protects Factor VIII) → prolonged aPTT
- PT is normal because the extrinsic pathway is unaffected
Read this in Haemostasis & Coagulation: Platelet Function & Bleeding Disorders
Von Willebrand Disease (vWD)
- Most prevalent inherited bleeding disorder worldwide — affects 1–3% of population
- Commonest cause of bleeding in children investigated for epistaxis and women investigated for menorrhagia
- Blood group O individuals have lower baseline vWF → ↑ susceptibility
- Qualitative OR quantitative defect in vWF
- vWF sources: endothelial cells (stored in Weibel-Palade bodies) + megakaryocytes (stored in α granules)
Read this in Platelet Disorders
What our notes say
- You are informed that there is a history of von Willebrand disease in her family and she shows you a registration card from when she was 6 years old on which a VWF antigen level of 0.42 iu/ml is documented. (Haematology paper 1 Past Papers — Past Paper Questions & Answers)
- Von Willebrand disease is the most common inherited bleeding disorder in the general population. (Haemostasis & Coagulation: Platelet Function & Bleeding Disorders)
- A girl with joint bleeds and prolonged venepuncture bleeding fits severe (type 3) von Willebrand disease. (Internal Medicine End of Semester CAT (MBChB 4): haematology and renal, 30 MCQs )
- Von Willebrand factor antigen and activity - normal, excluding von Willebrand disease (Haematology End of Year 2025 Past Paper — MKU MBML 3200 Questions & Answers)
- Cryoprecipitate - rich in fibrinogen, factor VIII, von Willebrand factor and factor XIII; for hypofibrinogenaemia, DIC, von Willebrand disease and massive haemorrhage protocols (Haematology End of Year 2025 Past Paper — MKU MBML 3200 Questions & Answers)
- Platelet aggregation studies — response to ADP, collagen, ristocetin; ristocetin-induced aggregation abnormal in von Willebrand disease and Bernard-Soulier syndrome (Renal, Liver, Cardiac & Adrenal Function Tests for Year 3)
Study material on Von Willebrand Disease
- Haemostasis & Coagulation: Platelet Function & Bleeding Disorders
- Platelet Disorders
- Haematology End of Year 2025 Past Paper — MKU MBML 3200 Questions & Answers
- Renal, Liver, Cardiac & Adrenal Function Tests for Year 3
- Haematology Exam 2025: MCQs on Anaemia, Leukaemia & Clotting
- Dr. Orata Haematology MCQs: Past Paper Questions & Answers
Practice questions on Von Willebrand Disease
Main abnormality in von Willebrand disease?
- Platelet deficiency
- Lack of von Willebrand factor
- Factor X deficiency
- Excess fibrinogen
Answer: B. vWD is the most common inherited bleeding disorder, caused by absent or dysfunctional vWF protein. ---
Von Willebrand disease is characterized by?
- Prolonged PT only
- Prolonged aPTT only
- Prolonged bleeding time and aPTT
- Prolonged PT and aPTT
Answer: C. vWF is needed for platelet adhesion (bleeding time) and carries Factor VIII (aPTT). ---
In type 1 Von Willebrand disease, laboratory findings show
- Prolonged PT, normal APTT
- Normal PT, prolonged APTT, reduced vWF antigen and activity
- Thrombocytopenia with prolonged PT
- Normal all coagulation tests
Answer: B. Reduced vWF quantity causes mildly prolonged APTT (low Factor VIII), prolonged bleeding time, and reduced ristocetin cofactor activity. ---
Von Willebrand disease is characterized by ---
- Prolonged PT only
- Prolonged aPTT only
- Prolonged bleeding time and aPTT vWF deficiency impairs platelet adhesion (↑bleeding time) and stabilizes Factor VIII (↑aPTT).
- Prolonged PT and aPTT
- Normal coagulation tests Answer:
Answer: A.
What is the main abnormality in von Willebrand disease? ---
- Platelet deficiency
- Lack of von Willebrand factor
- Factor IX deficiency
- Excess fibrinogen Answer:
Answer: A.