Haemophilia

Question 1

what is it?

Answer 1

• An X-linked, hereditary disorder in which abnormally prolonged bleeding recurs episodically at one or a few sites on each occasion
• Haemophilia A (factor VIII deficiency) – five times as common as
• Haemophilia B (factor IX deficiency)

Question 2

pathophysiology

Answer 2

• No abnormality of primary Haemostasis
• Bleeding from medium to large blood vessels
• Mild moderate and severely affected families depending on factor VIII/IX level

Question 3

what are the clinical presentations?

Answer 3

• Recurrent Haemarthroses
• Recurrent soft tissue bleeds – bruising in toddlers
• Prolonged bleeding after dental extractions, surgery and invasive procedures

Question 4

how to clinically approach bleeding disorders?

Answer 4

History
o	Bleeding/bruising
o	Duration (?life-long)
o	Previous surgery/dental extractions
Drug History
Family History
Examination