Haemophilia Flashcards Preview

Medicine Phase 2a Haematology > Haemophilia > Flashcards

Flashcards in Haemophilia Deck (14)
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1
Q

Inheritance pattern

A

X-linked recessive

2
Q

What is Haemophilia

A

X-linked recessive bleeding disorder

3
Q

Types of haemophilia

A

A and B

4
Q

Causes of Haemophilia A

A

Deficiency of factor VIII

5
Q

Causes of Haemophilia B

A

Deficiency of factor IX

6
Q

Signs and symptoms of Haemophilia A/B

A

Depend on disease severity
Bleeding is main feature and is prolonged, resulting in the need for investigations to uncover the cause
Positive family history may tailor diagnosis

7
Q

Investigations of Haemophilia A

A

Low factor VIII levels (lower this is, the more severe)
Coagulation factor assay
Increased PTT but not PT

8
Q

Conservative treatment of Haemophilia A

A

Patient and parent education
Genetic counselling and testing
Avoid anticoagulant medication e.g. NSAIDs, Warfarin, Aspirin

9
Q

Medical treatment of Haemophilia A

A

Mild - desmopressin

Severe - require IV replacement with plasma concentrate factor VIII

10
Q

Complications of Haemophilia A

A

Patients immune system may start to reject the IV plasma concentrate factor VIII by making inhibitors
Joint destruction by recurrent bleeding

11
Q

Investigations of Haemophilia B

A

Low factor IX levels (lower is more severe)
Coagulation factor assay
Increased PTT but normal PT

12
Q

Conservative treatment of Haemophilia B

A

Patient and parent education
Genetic counselling and testing
Avoid anticoagulant medication e.g. NSAIDs, warfarin, aspirin

13
Q

Medical treatment of Haemophilia B

A

IV infusion of factor IX

14
Q

Complications of Haemophilia B

A

Joint destruction by recurrent bleeding