Bleeding disorders

Question 1

What is haemophilia?

Answer 1

Bleeding disorder due to deficiency of clotting factors

X linked recessive

Question 2

What clotting factor is deficiency in haemophilia A?

Answer 2

Factor VIII

Question 3

What clotting factor is deficient in haemophilia B?

Answer 3

Factor IX

Question 4

What are the classifications of haemophilia?

Answer 4

Mild
Moderate- don’t tend to bleed spontaneously
Severe- regular spontaneous bleeds

Question 5

What is the severity of haemophilia dependent on?

Answer 5

Residual coagulation factor activity

Question 6

What are the features of haemophilia?

Answer 6

Haemarthrosis- esp into hinge weight hearing joints i.e. knee
Muscle haematoma
CNS bleeding
Retroperitoneal bleeding
Post surgical bleed

Question 7

How is haemophilia diagnosed?

Answer 7

Clinical
Genetic analysis
Prolonged APTT and normal PT
Reduced factor VIII or IX

Question 8

What is the pharmacological management of haemophilia?

Answer 8

Coagulation factor replacement
Desmopressin
Tranexemic acid
Analgesia

Question 9

What are the general treatment measures for haemophilia?

Answer 9

Splints

Physio

Question 10

What are the surgical treatment options for haemophilia?

Answer 10

Synovectomy
Joint replacement
Haematoma aspiration

Question 11

When are coagulation factor replacements given?

Answer 11

Prophylactically in severe disease

During bleed

Question 12

What are the complications of haemophilia?

Answer 12

Synovitis
Chronic haemophilic arthropahy
neuromuscular complications
Other sequelae of bleeding

Question 13

What are the complications of management of haemophilia?

Answer 13

Desmopressin- MI, hyponatraemia in babies

Development of inhibitors- common in those receiving factor VIII

Question 14

What is von Willebrand disease?

Answer 14

Common platelet type bleeding disease of variable severity

Autosomal inheritance

Question 15

What are the types of von Willebrand?

Answer 15

1= quantative
2= mutation causing poor function
3= complete deficiency

Question 16

What is the presentation of von Willebrand?

Answer 16

Menorrhagia
Epistaxis
Platelet type bleeding

Question 17

What is the management of von Willebrand?

Answer 17

Tranexemic acid for mild bleeding
vWF concentrate or desmopressin
OCP in women- prevent menorrhagia

Question 18

What is thrombocytopenia?

Answer 18

Low platelets

Question 19

What causes thrombocytopenia?

Answer 19

Decreased prodction- marrow failure, marrow infiltration, sepsis
Increased destruction- immune ITP, DIC, HUS, hypersplanism

Question 20

What are the clinical features of thrombocytopenia?

Answer 20

Petechia, esp on legs
Ecchymosis- bruising
Mucosal bleeding
Rarely CNS bleeding

Question 21

What are the precipitants of ITP?

Answer 21

Infection- esp viral
Drug induced
Lymphoproliferative disorders

Question 22

What is the management of ITP?

Answer 22

Steroids
IV Ig
AntiD immunoglobulin
Thrombopoetin analogues
Splenectomy if chronic

Question 23

How does liver failure cause bleeding disorders?

Answer 23

Failure to produce factors I, II, V, VII, VIII, IX, X, XI

1,2,5,7,8,9,10,11

Question 24

What are the clinical features of liver failure?

Answer 24

Bleeding from structural lesions e.g. varies
Jaundice
Itch
Anaemia

Question 25

How is liver failure bleeding diagnosed?

Answer 25

Prolonged PT and APTT

Reduced fibrinogen

Question 26

What is the treatment of liver failure bleeding?

Answer 26

Vit K

Replacement FFP

Question 27

What is haemorrhagic disease of the newborn?

Answer 27

Immature coagulation systems caused by vit K defieiency

Question 28

How is haemorrhage disease of the newborn prevented?

Answer 28

Vit K at birth