Bleeding Disorders Flashcards
Normal haemostastic response
Primary- Platelet Plug formation, platelets, vWF, endothelial wall
Secondary- Fibrin plug formation
What is haemorrhagic diathesis?
Unusual susceptibility to bleed
- Any quantitative or qualitative abnormality inhibition of function
- Due to platelets, vWF, coagulation factors
Points to ask in bleeding disorders bleeding history?
Has patient got a bleeding disorder, How severe is it?, pattern of bleeding, Congenital or acquired, Mode of inheritance
Key points to ask about with suspected bleeding disorder to ascertain how severe?
How appropriate? Bruising, Epistaxis, Post surgical bleeding (how long? Outcome? Dental surgery, tonsillectomy?) Menorrhagia, Post-partum haemorrhage, Post-trauma, Spontaneously?
If due to platelets what types of bleeds are you likely to see?
Mucosal Epistaxis Purpura Menorrhagia GI
If due to coagulation factors what types of bleeds are you likely to see?
Articular
Muscle haematoma
CNS (intracranial bleed)
Questions to ask to assess if bleeding disorder is congenital or acquired
Previous episodes ?
Age at first visit for bleed ?
Previous surgical challenges ?
Associated history ?
Types of inheritance of haemophilia
X-linked
What is haemophilia?
Genetic disorder impairing body’s function to make clots
Severity of bleeding depends on the residual coagulation factors activity:
<1% severe
1-5% moderate
5-30% mild
Presentation of haemophilia
Haemoarthrosis: Bleeding into joint
Muscle Haematoma: Bleeding into muscle
CNS bleeding: Less commonly seen
Retroperitoneal bleeding
Post-surgical bleeding
Difference between haemophilia A and B
A=deficiency in factor 8
B=deficiency in factor 9
Haemophilia clinical complications
Synovitis,
Chronic haemophilic arthropathy,
Neurovascular compression (eg compartment syndromes),
Other sequelae of bleeding (eg stroke)
Haemophilia diagnosis
Clinical, Prolonged APTT, Normal PT, Reduced factor 8 or 9, Genetic analysis
Haemophilia treatment of bleeding diathesis
Coagulation factor replacement, DDAVP (causes release of vWF) Tranexamic acid, Prophylaxis, Gene therapy
Treatment of haemophilia to prevent bleeds
Splints, Physiotherapy, Analgesia, Synovectomy, Joint replacement