Common Bleeding Disorders

Question 1

What is Haemophilia?

Answer 1

=> 2 types:

• Haemophilia A (deficiency of factor VIII)
• Haemophilia B (deficiency of factor IX)

X linked recessive disorder of coagulation

Question 2

What are the clinical features of Haemophilia?

Answer 2

• Haemoarthroses, haematomas
• Prolonged bleeding after surgery or trauma

=> Blood tests:

• Prolonged APTT
• Bleeding time, thrombin time, prothrombin time is normal

Question 3

What is Von Williebrand disease?

Answer 3

• Most common inherited bleeding disorder
• Inherited in autosomal dominant fashion
• vWF promotes platelet adhesion in cases of endothelial damage, and in von Williebrand disease this is reduced

=> Different types of von Williebrand disease:

• Type 1 (partial reduction of vWF)
• Type 2 (abnormal form of vWF)
• Type 3 (total absence of vWF)

=> Type 3 is inherited as a autosomal recessive trait

Question 4

What are the investigations in suspected Von Williebrand disease?

Answer 4

• Prolonged APTT
• Prolonged bleeding time
• Factor VIII levels may be reduced

Question 5

What is the management of Von Williebrand disease?

Answer 5

• Tranexamic acid for mild bleeding
• Desmopressin
• Factor VIII concentrate

Question 6

What is Disseminated Intravascular Coagulation?

Answer 6

• Processes of dysregulated coagulation and fibrinolysis, resulting in widespread clotting and bleeding

Question 7

What are the causes of DIC?

Answer 7

• Sepsis
• Malignancy
• Obstetric complications
• Trauma

Question 8

What do the blood tests of DIC show?

Answer 8

• Prolonged bleeding time
• Prolonged prothrombin time
• Prolonged APTT
• Presence of fibrin degradation products
• Schistocytosis (microangiopathic haemolytic anaemia)