Bleeding Disorders Flashcards
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?
Mucosal bleeds Epistaxis Purpura & petechiae Menorrhagia GI bleeds
What kind of bleeds would happen in someone with a coagulation factor problem?
Articular bleeds
muscle Haematoma
CNS bleeds
How would you investigate a patient’s history to determine if its a congenital or acquired bleeding disorder?
FH Previous episodes Age at first event Previous surgical challenges Associated History
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: - Haemarthrosis - 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
Genetic Tests
How can we manage acute bleeding events in haemophilia?
Coagulation Factor replacement
Desmopressin
Tranexamic Acid
What can we give patients as prophylaxis for haemophilia?
Regular Coagulation Factor Injs Splints & Physio Analgesia Synovectomy Joint Replacement
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
