Bleeding Disorders

Question 1

What is haemorrhagic diathesis?

Answer 1

An unusual susceptibility to bleeding

Question 2

What would you want to know in a history of bleeding?

Answer 2

• Bruising
• Epistaxis
• Post surgical bleeding
• Menorrhagia
• Post-partum haemorrhage
• Post-trauma

Question 3

What patterns of bleeding would make a disorder of the platelets more common?

Answer 3

• Mucosal
• Epistaxis
• Purpura
• Menorrhagia
• GI bleeds

Question 4

What patterns of bleeding would make a coagulation factor disorder more common?

Answer 4

• Articular
• Muscle haematoma
• CNS

Question 5

What factors make a congenital disorder more likely?

Answer 5

• Previous episodes
• The age at first event
• Previous surgical challenges
• Associated history

Question 6

Describe the features of haemophilia A and B

Answer 6

• X linked
• Identical phenotypes
• Severity of bleeding depends on the residual coagulation factor activity
• Severe <1%
• Moderate 1-5%
• Mild 5-30%

Question 7

What are the clinical features of haemophilia?

Answer 7

• Haemarthrosis (hinge joints do much worse than ball and socket joints)
• Muscle haematoma
• CNS bleeding
• Retroperitoneal bleeding
• Post surgical bleeding

Question 8

What are the complications of haemophilia?

Answer 8

• Synovitis
• Chronic haemophilic arthropathy
• Neurovascular compression
• Sequelae of bleeding (stroke etc.)

Question 9

How can haemophilia be diagnosed?

Answer 9

• Clinical
• Prolonged APTT
• Normal PT
• Reduced FVIII or FIX
• Genetic analysis

Question 10

How can the bleeding diathesis of haemophilia be treated?

Answer 10

• Coagulation factor replacement FVIII/IX
• DDAVP (desmopressin)
• Tranexamic acid
• Prophylaxis in severe haemophilia
• ?Gene therapy

Question 11

How can haemophilia be managed?

Answer 11

• Splints
• Physio
• Analgesia
• Synovectomy
• Joint replacement

Question 12

What complications can haemophilia treatment have?

Answer 12

• Viral infection
• Inhibitors: anti FVIII antibodies etc.
• DDAVP can cause MI and hyponatraemia

Question 13

Describe the features of von Willebrand disease

Answer 13

• Variable severity
• Autosomal
• Platelet type bleeding

Question 14

List the three different types of Von Willebrand Disease

Answer 14

• Type 1: quantitative deficiency
• Type 2: qualitative deficiency determined by the site of the mutation
• Type 3: severe (complete) deficiency

Question 15

How can Von Willebrand disease be treated?

Answer 15

• vWF concentrate or DDAVP
• Tranexamic acid
• Topical applications
• OCP etc.

Question 16

Give examples of acquired bleeding disorders

Answer 16

• Thrombocytopenia
• Liver failure
• Renal failure
• DIC
• Those caused by drugs

Question 17

What are the causes of thrombocytopenia?

Answer 17

• Decreased production: marrow failure, aplasia and infiltration
• Increased consumption: immune ITP, non immune DIC and hypersplenism

Question 18

What is the clinical presentation of thrombocytopenia?

Answer 18

• Petechia
• Ecchymosis
• Mucosal bleeding
• Rare CNS bleeding

Question 19

What are the causes of immune thrombocytopenia?

Answer 19

• Infections
• Collagenosis
• Lymphoma
• Drug induced
• Blood isolated

Question 20

How can ITP be treated?

Answer 20

• Steroids
• IgG
• Splenectomy
• Thrombopoietin analogues (eltrombopag and romiplostim)

Question 21

What effect does liver failure have on clotting and how can it be treated?

Answer 21

• It causes a reduction in the factors
• Prolonged PT, APTT and reduced fibrinogen
• Cholestasis and vit K factor deficiency
• Managed by replacement FFP and vitamin K

Question 22

What is haemorrhagic disease of the newborn and how can it be treated?

Answer 22

• Immature coagulation systems
• Vitamin K deficient diet
• Fatal and incapacitating haemorrhage
• Preventable by administration of vitamin K at birth