Bleeding Disorders

Question 1

What is the normal haemostatic response to bleeding?

Answer 1

Primary - platelet plug, vWF

Secondary - fibrin plug

Question 2

Haemorrhagic diathesis is caused by abnormalities in what?

Answer 2

Platelets
vWF
Coaglation factors

Question 3

What are common in a history of bleeding?

Answer 3

Bruising 
Epistaxis
Post-surgical bleed
Menorrhagia
Post-partum haemorrhage
Post-trauma haemorrhage

Question 4

What is an abnormal platelet pattern of bleeding?

Answer 4

Mucosal
Epistaxis 
Purpura
Menorrhagia
GI bleeds
Patechiae (don't blanche)
Bruising

Question 5

What is a coagulation factor pattern of bleeding?

Answer 5

Articular (knee or ankle)
Muscle haematoma
CNS bleeds
Hot, tender, swollen joint

Question 6

How can you differentiate congenital and acquired bleeding disorders?

Answer 6

Previous episodes
Age of first event
Previous surgical issues
Associated history

Question 7

What is the cause of Haemophilia A and B?

Answer 7

X-linked gene causing lack of factor VIII (A) or IX (B)

Question 8

Degree of bleeding/severity in Haemophilia A/B depends on what?

Answer 8

Residual coagulation factor activity

Question 9

Clinical features of Haemophilia?

Answer 9

Haemarthrosis (hinge joints)
Muscle hematoma
CNS bleed
Retroperitoneal bleed
Post-surgical bleed

Question 10

What complications are associated with Haemophilia?

Answer 10

Synovitis
Chronic Haemophilic Arthropathy
Compartment syndrome
Stroke

Question 11

How is Haemophilia diagnosed?

Answer 11

Prolonged APTT
Normal PTT
Reduced Factor VIII or IX
Gene analysis
CVS/Amniocentesis in carrier mother

Question 12

How is Haemophilia bleeding managed?

Answer 12

Missing factor replaced
Tranexamic acid
Desmopressin
Prophylaxis

Question 13

How is Haemophilic Arthritis treated?

Answer 13

Splints 
Physiotherapy 
Analgesia 
Synovectomy 
Joint replacement

Question 14

How does von Willebrand disease present?

Answer 14

Platelet type bleeding (mucosal)

Autosomal (1 in 200)

Question 15

Outline von Willebrand disease types

Answer 15

Type 1: minor
Type 2: (qualitative) determined by site of mutation
Type 3: complete deficiency

Question 16

How is von Willebrand disease managed?

Answer 16

vWF concentrate
Desmopressin
Tranexamic acid
Oral contraceptive (to control period)

Question 17

Name 3 acquired bleeding disorders?

Answer 17

Thrombocytopenia
Liver failure
Renal failure
DIC 
Antiplatelet/anticoagulant drugs

Question 18

What are the causes of decreased platelet production in thrombocytopenia?

Answer 18

Marrow failure
Aplasia
Infiltration

Question 19

What are the causes of increased platelet destruction in thrombocytopenia?

Answer 19

Immune thrombocytopenic purpura
Non-immune DIC
Hypersplenism

Question 20

How does Thrombocytopenia present?

Answer 20

Petechiae
Ecchymosis - bruising
Mucosal bleeding
CNS bleeding

Question 21

How is Immune thrombocytopenic purpura managed?

Answer 21

Steroids
IV IgG
Splenectomy
Thrombopoeitin analogues

Question 22

How does Liver failure associated bleeding disorder present?

Answer 22

Prolonged PT, APTT
Reduced Fibrinogen
Cholestasis
Variceal bleeding

Question 23

How is Liver failure associated bleeding disorder managed?

Answer 23

Vitamin K

Replacement FFP

Question 24

How is Haemorrhagic Disease of the Newborn managed?

Answer 24

Vitamin K at birth

Question 25

Spontaneous bleeding only occurs in what?

Answer 25

SEVERE haemophilia

Question 26

DDAVP is associated with what complications?

Answer 26

Risk in Hyperlipidemic/MI patients

Hyponatremia in babies

Question 27

What is the difference between qualitative and quantitative vW disease?

Answer 27

Quantititive - “amount of factor missing”

Qualitative - “variation depends on location of mutation”

Question 28

What are the causes of DIC (Disseminated Intravascular Coagulation)?

Answer 28

Massive trauma, infection, surgery, pregnancy complications etc