Flashcards in Bleeding Disorders Deck (18)
What in a history would suggest a bleeding disorder rather than a normal bleed?
Large post-surgical bleeding or menorrhagia, frequent nosebleeds or bruising etc
Innapropriate bleeding e.g. after a vaccination or unprovoked
The pattern of bleed is important as it indicates what kind of disorder you may have. How would a platelet deficiency bleed?
Purpura & petechiae
What kind of bleeds would happen in someone with a coagulation factor problem?
How would you investigate a patient's history to determine if its a congenital or acquired bleeding disorder?
Age at first event
Previous surgical challenges
What is the most common heritable coagulation factor disorder?
Haemophilia A & B
A = Factor 8
B = Factor 9
Lumped together because the phenotype is identical
What determines severity of Haemophilia?
Residual Coagulation Factor Activity
Severe = <1%
Mod = <5%
Mild = <30%
How does a person with haemophilia present?
Obviously with loads of bleeds (spontaneously, excessively or on little stimulation)
Bleeds will be:
- Muscle Haematoma
- CNS bleeds
- Peritoneal Bleeds
- Post-surgical bleeds
Other than obviously bleeding loads, what's the big issue with haemophilia?
Patients tend to bleed into hinge, weight bearing joints i.e. knee
This leads to Chronic Haemophilic Arthropathy, with most patients developing end stage joint disease by 30 if they're not treated properly
How do we go about diagnosing a haemophilia if we suspect it from history?
FBC + aPTT + PT
Coagulation Facotr 8/9 Assay
How can we manage acute bleeding events in haemophilia?
Coagulation Factor replacement
What can we give patients as prophylaxis for haemophilia?
Regular Coagulation Factor Injs
Splints & Physio
Treating Haemophilia comes with it's own complications, what the majorones?
1) Viral infections e.g. HIV from donated coagulation factors (rare today as we use recombinant ones)
2) Development of Anti-factor Abs
3) Desmopressin SEs
How do haemophilia patients develop Anti-factor Abs?
Patients with a null mutation (meaning they completely lack the Factor, mostly in Factor 8 aka haemophilia A) i.e. Severe Haemophilia
When they're eventually exposed to the Factor it's foreign to them so their bodies produce Anti-Factor8 Antibodies
What are the complications of Desmopressin?
Can cause MI so contraindicated in IHD
Can cause Hyponatraemia in infants --> Seizures so contraindicated under 3 yrs
What is the most common platelet disorder?
von Willebrand's Disease
1 in 200, making it the most common heritable bleeding disorder by far
How are haemophlia & von Willebrand's Disease inherited?
Haemophilia = X-linked
von Willebrand's = Autosomal
What are the types of von Willebrand's?
1 = Quantitative deficiency
2 = Qualitative deficiency
3 = Complete deficiency