Bleeding disorders Flashcards
What points on a history are useful in determining if someone has a bleeding disorder?
Bruising
Epistaxis
Post-surgical bleeding
- dental surgery
- circumcision
- tonsillectomy
- appendicectomy
Menorrhagia
Post-partum haemorrhage
Post-trauma
What patterns of bleeding are seen in patients with ‘platelet type’ bleeding?
eg thrombocytopenia, platelet dysfunction, vWF deficieny
Mucosal bleeding (hallmark of thrombocytopenic bleeding):
- epistaxis
- purpura / petechiae
- menorrhagia
- GI bleeds
Post-surgical bleeding
What patterns of bleeding are seen in coagulopathic bleeding?
eg Haemophilia A & B
Coagulopathic bleeding characterised by:
- Articular bleeding - esp ankle, knee, elbow
- Muscle haematoma
- CNS bleeds
Also have post surgical/intervention bleeds
What are haemophilia A and B?
How are they inherited?
Which is more common?
Bleeding disorders caused by a deficiency in Factor VIII and Factor IX respectively
Both Haemophilia A and B have identical phenotypes and are X-linked (male disease)
Haemophilia A (Factor VIII deficiency) is more common
What determines the severity of bleeding in Haemophilia A & B?
Severity of bleeding depends on residual coagulation factor activity…
Severe haemophilia = residual activity <1%
Moderate = 1-5%
Mild = 5-30%
What are the clinical features of haemophilia?
Haemoarthrosis - bleeding into joints
Muscle haematoma
CNS bleeds
Retroperitoneal bleeding
Post-surgical bleeding
What are the clnical complications of haemophilia?
Synovitis - following haemoarthropathy
Chronic haemophilic arthropathy
Neurovascular compression (compartment syndrome)
Other sequelae of bleeding
How is a diagnosis of Haemophilia A or B made?
For the blood tests - give the abnormality expected if haemophilia is present
1) Clinical history
2) Routine coagulation factor testing:
- prolonged APTT - activated partial thromboplastin time
- normal/prolonged PT - prothrombin time
- then - FVIII / FIX testing
3) Genetic testing
Describe the treatment of haemophilia A/B
Coagulation factor replacement (recombinant products) - FVIII/FIX
Desmopressin (DDAVP) - to stop bleeding in mild Haemophilia A (also used vWF deficiency)
Tranexamic acid - reduces rate of fibrinolysis
Prophylaxis - in severe haemohilia
Gene therapy coming soon
How are the complications of haemophilia such as arthropathies managed?
Splints
Physiotherapy
Analgesia
Synovectomy
Joint replacement
What are the complications of haemophilia treatment?
Transfusion related infections:
- HIV
- Hep - HBV, HCV
- vCJD
Inhibitor development:
- anti-FVIII antibody development
- rare in haemophilia B
Complications of desmopressin therapy:
- MI & TIA
- Hyponatraemia (low sod) in babies
What are contraindications to desmopressin therapy for haemophilia?
High CVS risk - to avoid MI & TIA
Age <3 - to avoid hyponatraemia
What is von Willebrand’s disease?
Describe the inheritance and how common it is
What gender is affected by it?
Common bleeding disorder in which there is qualititive and/or quantitive defects of vWF
Very common
Autosomal (usually dominant) inheritance
Affects both men and women
What pattern of bleeding is seen in von Willebrands disease?
How severe is the bleeding in vWD?
Platelet bleeding pattern - ie bleeding of mucosal membranes
(epistaxis, purpura etc)
Bleeding severity varies between individuals - with some having severe problems and some having mild
What are the types of vWD?
Type 1 - quantitative deficiency
Type 2 - qualitative deficiency:
A, B, M, N qualitative subtypes depending on site of mutation
Type 3 - severe (complete) deficiency
Does Liver disease cause increased risk of bleeding or increased risk of coagulation?
Both
Go look at the liver disease stuff
