bleeding disorders Flashcards
What points do you need think about in a history of bleeding disorders?
○ Has the patient actually got a bleeding disorder ○ How severe is the disorder? ○ Pattern of Bleeding ○ Congenital or aquired ○ Mode of inheritance
What should you ask when you are taking a history of the bleeding?
○ Bruising ○ Epistaxis ○ Post-surgical bleeding - Dental Surgery - Circumcision - Tonsillectomy - Appendicectomy ○ Menorrhagia ○ Post-partum haemorrhage ○ Post-trauma ○ Family history
What should you concider to assertain the severity of the bleeding?
○ If you are stabbed in the heart bleeding is pretty normal (you have a hole in your heart)
○ If you are bleeding getting a vaccine then it is not appropriate
○ If you are doing nothing and start bleeding into your knees then that is severe
What should be asked when trying to assertain the pattern of bleeding?
○ Platelet type (or VWF) - Mucosal - Epistaxis (recurrent + doesn’t stop) - Purpura (non-blanching spots) - Menorrhagia - GI ○ Coagulation factor - Articular - Muscle Haematoma - CNS
What should be asked to ascertain whether the bleeding is congenital or aquired?
○ Previous Episodes ? ○ Age at first event ○ Previous surgical challenges ○ Associated History ○ Hereditary disorders - Family members with similar history - Sex
What is haemophilia A and B?
○ X-linked
○ Identical phenotypes
○ Severity of bleeding depends on the residual coagulation factor activity
What is the epidemiology of haemophilia A and B?
○ 1 in 10,000 and 1 in 60,000
- <1% Severe (can start bleeding without provocation)
- 1-5% Moderate
- 5-30% Mild
What are the clinical features of haemophilia?
- Haemarthrosis
- Muscle haematoma
- CNS bleeding
- Retroperitoneal bleeding
- Post-surgical bleeding
What are the clinical complications of haemophilia?
- Synovitis
- Chronic Haemophilic Arthropathy
- Neurovascular compression (compartment syndromes)
- Other sequelae of bleeding (Stroke)
How is haemophilia diagnosed?
- Clinical
- Prolonged APTT
- Normal PT
- Reduced FVIII or FIX
- Genetic analysis
How is bleeding diathesis managed?
□ Coagulation factor replacement FVIII/IX
□ Now almost entirely recombinant products
□ DDAVP
□ Tranexamic Acid
□ Emphasis on prophylaxis in severe haemophilia
□ Gene therapy? (future)
How is haemophilia managed?
□ Splints
□ Physiotherapy □ Analgesia □ Synovectomy □ Joint replacement
What is Von Willebrand disease?
- Common (1 in 200)
- Variable severity
- Autosomal
- Platelet Type bleeding (mucosal)
- Quantitative and qualitative abnormalities of vWF
What are the different types of Von Willebrand disease?
□ Type 1 quantitative deficiency
□ Type 2 (A,B,M,N) qualitative deficiency determined by the site of mutation in relation to vWF function
□ Type 3 severe (complete) deficiency
How is Von Willebrand disease managed?
□ vWF concentrate or DDAVP
□ Tranexamic Acid
□ Topical applications
□ OCP etc
