Von Willebrand Disease | Presentation | Diagnosis | Classification | Management
Channel: 2.5 Minutes Medicine
Date: 2022-10-25

Von Willebrand Disease (vWD)

is caused by a decreased level of functioning von Willebrand factor, which plays a crucial

role in primary hemostasis, Most forms of vWD follow an inherited autosomal dominant pattern that affects males and females equally. vWD is the most common inherited bleeding disorder.

Pathophysiology: 
✓ Qualitative defect or quantitative

deficiency of vWF.

 ✓ VWF needed for platelet adhesion and aggregation, and acts as a chaperone for Factor VIII, extending its half life in circulation. Thus, VWD affects both primary and secondary hemostasis.

clinically presented as :
✓ Frequent nosebleeds.

✓ Oral mucosa bleeding.

✓ GI bleeding.

✓ Heavy menstrual cycles.

✓Easy bruising.

✓ Prolonged bleeding after surgery or trauma.


differential Diagnosis:
✓ Hemophilia A, B, C.

✓ Bernard Soulier syndrome.

✓ Platelet disorders.

 ✓ Antithrombotic therapies.

✓ Trauma: accidental or inflicted.


investigations that need to be carried are; 

Personal and family history of

bleeding with consistent labs:
 ✓ CBC, platelets, PTT.

✓ Plasma vWF antigen levels.
 ✓ vWF:Ristocetin cofactor. activity (determine how well vWF binds platelets).

✓  Factor 8 levels.

supportive management:
✓ Stop the bleeding.

✓  Ensure hemodynamic stability.

✓ Minimize trauma.

✓ Avoid medications that could worsen bleeding

symptoms.

✓ Only -10% require long-term prophylactic therapy.

#health #bloodline #fitness #bloodline #coagulation #bleeding #epistaxis