Bleeding disorder - Von Willebrand Disease Flashcards
How common is von willebrand’s disease?
The most common cause of haemophilia
What is haemophilia?
Abnormal bleeding
What are most von willebrand diseases caused by?
Autosomal dominant inheritance (NOT on the X chromosome)
Type 3 is autosomal recessive
What are the types of von willebrand diseases?
Type 1 - most common, mild.
Type 2 - enough VWF but the quality is poor, mild.
Type 3 - very severe, theres basically no VWF.
What is the role of von willebrand factor?
Promotes aggregation of platelets to each other and the damaged epithelium
Is a factor VIII carrier
What are the most common presentations of types 1 and 2?
Usually asymptomatic or mild.
MUCOSAL bleeding: Easily bruised Unusually heavy/prolonged bleeding Menorrhagia Post surgical bleeding Family history Epistaxis Bleeding of gums
What is the most common presentation of type 3?
Joint + muscles haematomas
GI bleeding
When is acquired von willebrand diseases seen?
Often after an autoimmune condition arises.
What are causes of acquired von willebrand diseases?
Autoimmune diseases
SLE
Valproic acid
Ciprofloxacin
What are tests to diagnose von willebrand disease?
No gold standard test.
Prolonged bleeding time Platelet count - normal Prothrombin time - normal Activated partial thromboplastin time (aPTT) - increased WWF Antigen Family history Clinical signs
What is the treatment of von willebrand disease?
Tranexamic acid - mild bleeding (especially in women with menorrhagia)
DDAVP - increases levels of VWF
VWF infusion (+ factor VIII given alongside)
What does tranexamic acid do?
antifibrinolytic - blocks the breakdown of clots.
What treatment is given for menorrhagia?
Tranexamic acid
Combined oral contraceptive
Mirena coil
Hysterectomy
When is DDAVP (desmopressin) not given?
For type 3 Von willebrand disease - because theres a complete deficiency
They’re given IV VWF with IF factor VIII.
What is the most characteristic type of bleeding?
Platelet type bleeding (Mucosal)
