Bleeding Disorders

Question 1

What is the normal haemostatic response?

Answer 1

• primary
• platelet plug formation
• platelets
• von Willebrand Factor (vWF)
• wall
• secondary
• fibrin plug formation

Question 2

What is haemorrhagic diathesis?

Answer 2

• any quantitive or qualitative abnormality
• inhibition of function
• platlets
• vWF
• coagulation factors

Question 3

What are different patterns of bleeding?

Answer 3

• platelet type
• mucosal
• epistaxis
• purpura
• menorrhagia
• GI
• coagulation factor
• articular
• muscle haematoma
• CNS

Question 4

What are features of haemophilia A and B?

Answer 4

• X-linked
• identicle phenotypes
• 1 in 10,000 + 1 in 60,000
• severity of bleeing depends on residual coagulation factor activity
• < 1% severe
• 1-5% moderate
• 5-30% mild

Question 5

What are signs of haemophilia?

Answer 5

• haemarthrosis
• muscle haematoma
• CNS bleeding
• retroperitoneal bleeding
• post surgical bleeding

Question 6

What are complication of haemophilia?

Answer 6

• synovitis
• chronic haemophilic arthropathy
• neurovascular compression
• compartment syndomes
• other sequela of bleeding
• stroke

Question 7

What are investigations for haemophilia?

Answer 7

• clinical
• prolonged APTT
• normal PT
• dec. FVIII or FIX
• genetic analysis

Question 8

What is the treatment for haemophilia bleeding diathesis?

Answer 8

• coagulation factor replacement FVIII/IX
• recombinant products
• DDAVP
• tranexamic acid
• prophylaxis in severe haemophilia
• gene therapy

Question 9

What is the treatment for haemophilia?

Answer 9

• splints
• physiotherapy
• analgesia
• synovectomy
• joint replacement

Question 10

What is treated as haemophilia complications?

Answer 10

• viral infections
• HIV
• HBV, HCV
• inhibitors
• anti FVIII Ab
• rare in FIX
• DDAVP
• MI
• hyponatraemia (babies)

Question 11

What are features of von Willebrand disease?

Answer 11

• common, 1 in 200
• variable severity
• autosomal
• platelet type bleeding (mucosal)
• quantative + qualitative abnormalities of vWF

Question 12

What are the types of von Willebrand disease?

Answer 12

• type 1- quantitative deficiency
• type 2 (A, B, M, N)- qualitative deficiency, determined by the site of mutation in relation to vWF function
• type 3- severe (complete) deficiency

Question 13

What is the treatment for von Willebrand disease?

Answer 13

• vWF concentrate/DDAVP
• tranexamic acid
• topical applications
• OCP, etc

Question 14

What are causes of aquired bleeding disorders?

Answer 14

• thrombocytopenia
• liver failure
• renal failure
• DIC
• drugs
• warfarin, heparin, aspirin, clopidogrel

Question 15

What is thrombocytopenia?

Answer 15

• dec. production
• marrow failure
• aplasia
• infiltration
• inc. consumption
• immune ITP
• non-immune DIC
• hypersplenism

Question 16

What are the signs of thrombocytopenia?

Answer 16

• petechia
• ecchymosis
• mucosal bleeding
• CNS bleeding (rare)

Question 17

What is immune thrombocytopenic pupura (ITP)?

Answer 17

• associated with; infection, collagenosis, lymphoma, drug induced
• blood isolated thrombocytopenia
• marrow
• steroid, IV IgG, splenectomy, thrombopoietin analogues

Question 18

What is haemorrhagic disease of the newborn?

Answer 18

• immature coagulation systems
• vitamin K deficient diet (ecp. breast)
• fatal + incapacitating haemorrhage
• completely [reventable by administration of vitamin K at birth