Haemophilia Flashcards
What type of condition is Haemophilia?
X-linked recessive disorder of coagulation
What type of inheritance does haemophilia have?
X-linked recessive
How many types of haemophilia are there?
2
Haemophilia A
Haemophilia B
Which type if haemophilia is more common?
Haemophilia A
What is haemophilia A due to?
Deficiency in factor 8
What is Haemophilia B due to?
Deficiency of factor 9
What is another name for haemophilia B?
Christmas disease
What are the main features of haemophilia?
- Hemarthrosis (bleeding into the joint)
- Muscle haematomas
- Prolonged bleeding after surgery or trauma
- CNS bleeding
When does haemophilia tend to present?
In early life
What is the main presentation of haemophilia?
Severe bleeding into soft tissue, joints and muscle
How is haemophilia A and B diagnosed?
Assay
How would happen to the APTT in severe haemophilia?
It would be prolonged
What would you do an assay for?
Specific assay for factor 8 and Factor 9
What is the management of haemophilia?
Recombinant coagulation factor 8/9 replacement
What are clinical complications for haemophilia?
- Synovitis (inflammation of membrane surrounding joint)
- Chronic haemophilic arthropathy
- Neurovascular compression- compartment syndromes
- Viral infection- HIV, Hep B and Hep C (transmitted by blood products)
