Bleeding Disorders

Question 1

Give two types of haemophilia and their cause .

What’s it inheritance pattern?

Answer 1

Haemophilia A
= factor 8 deficiency

Haemophilia B ( Christmas disease)
= factor 9 deficiency 

X-linked recessive -> affects males

Question 2

What at signs of bleeding disorders such as haemophilia?

Answer 2

Recurrent spontaneous bleeding into joints, muscles , soft tissue

• haemarthrosis ( bleeding into joint)
• haematomas
• mucous bleeding eg gum
• haematuria

In neonates

• intracranial
• post circumcision
• prolonged oozing from venepuncture

Question 3

In haemophilia , what would the PT ( prothrombin) and APTT ( activated partial thromboplastin time ) and the platelet count be?

Answer 3

Normal PT
Normal platelet
Prolonged APTT

Question 4

Which duet can be used to enable minor surgery or treat mild haemophilia A and von Willebrandt factor ?

Answer 4

Desmopressin= DDAVP

Stimulates endogenous release of F8 clotting activities and vWF

Question 5

How to treat acute bleeding in a patient with haemophilia ?

Answer 5

Factor replacement 8 or 9
IV

Also : attach to haemophilia centre !!!

Question 6

Functions of v willebrand factor

Answer 6

Fascilitates platelet adhesion to damages epithelium

Acts as carriers protein for FVII:C

Question 7

Clinically , how would you differentiate haemophilia from v willebrand disease?

What would specific blood tests reveal ?

Answer 7

Brushing and excessive bleeding and mucosal bleeding but no spontaneous bleeding and haemarthrosis uncommon

PT normal
APTT slight high or normal
Factor VIII:C slightly low or normal
Decreases VWF antigen

Question 8

What would you avoid in a patient with a bleeding disorder ?

Answer 8

IM injections
Aspirin
NSAIDS

Question 9

What’s DIC and what are causes ?

Answer 9

Disseminated intravascular coagulation

Systemic activation of clotting cascade causing haemorrhagic manifestations due to microthrombi, platelet consumption, complete clotting factor consumption

Sepsis 
Shock
Trauma 
Malignancies 
Haemolytic transfusion reaction 
Amniotic fluid embolism
Pre- eclampsia 
Acute pancreatitis

Question 10

What are signs of DIC?

Answer 10

Petechiae
Purpura
Haematuria 
Haematemesis 
AKI
purpura fulminans 
DVT
Stroke 
...

Question 11

How to manage a patient with DIC?

Answer 11

Treat cause
Blood transfusions
Heparin , activated Protein C
Don’t inhibit fibrinolysis ( with tranexamic acid )