Bleeding Disorders Flashcards
What in a history would suggest a bleeding disorder rather than a normal bleed? [4]
Large post-surgical bleeding
Menorrhagia, frequent nosebleeds
Inapropriate bleeding e.g. after a vaccination
Unprovoked bleeding
The pattern of bleed is important as it indicates what kind of disorder you may have. How would a platelet deficiency bleed? [4]
Mucosal, GI bleeds
Epistaxis
Purpura & petechiae
Menorrhagia
What kind of bleeds would happen in someone with a coagulation factor problem? [3]
Articular bleeds
muscle Haematoma
CNS bleeds
How would you investigate a patient’s history to determine if its a congenital or acquired bleeding disorder? [3]
FH
Previous episodes, age at first event
Previous surgical challenges
What is the deficiency in hemophilia A and B? [2]
Lumped together because the phenotype is identical
Mode of inheritance [1]
Haemophilia A & B
A = Factor 8
B = Factor 9
X-linked
What determines severity of Haemophilia? [3]
Residual Coagulation Factor Activity
Severe = <1%
Mod = <5%
Mild = <30%
Clinical features of hemophilia [5]
Sites affected [1]
Bleeds will be:
- Haemarthrosis
- Muscle Haematoma
- CNS bleeds
- Peritoneal Bleeds
- Post-surgical bleeds
Sites affected: weight bearing joints eg knees
Clinical features of hemophilia
If untreated, what can happen? [3] + [2]
A complication of treatment is alloantibody reaction. What can occur in null mutations?
Synovitis and chronic hemophilic arthropathy
Macrophages take up blood triggering inflammatory cytokine release
Drives degeneration of joint and loss of articular cartilage
Neurovascular compression
Other sequelae of bleeding eg stroke
Null mutations - anti factor 8 abs can be produced
Investigations- haemophilia [4]
Clinical - around walking age
Prolonged aPTT, Normal PT
Reduced Coagulation Factor 8/9 Assay
Genetic analysis
How can we manage acute bleeding events in haemophilia? [3]
Coagulation Factor replacement
Desmopressin/ DDAVP
Tranexamic Acid
Prophylaxis is key in management. What threshold of factor 8 will result in zero bleeds in a year? [1]
What can we prescribe patients as prophylaxis for haemophilia? [4]
Cannot let factor 8 fall below 2
Regular Coagulation Factor 8/9 Injs Tranexamic acid, DDAVP For the joints: Splints & Physio Analgesia Synovectomy or joint Replacement
What are the complications of Desmopressin? [2]
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?
Pattern of bleeding?
von Willebrand’s Disease
platelet type bleeding
How are haemophlia & von Willebrand’s Disease inherited?
Haemophilia = X-linked
von Willebrand’s = Autosomal dominant
What are the types of von Willebrand’s? [3]
1 = Quantitative deficiency 2 = Qualitative deficiency 3 = Complete deficiency
