Bleeding disorders Flashcards
After injury, what 3 processes halt bleeding?
Vasoconstriction
Gap plugging by platelets
Coagulation cascade
What 3 groups do disorders of haemostasis fall into?
Vascular defects
Platelet defects
Coagulation defects
What pattern of bleeding do coagulation disorders cause?
Delayed bleeding
What pattern of bleeding do vascular and platelet disorders cause?
Prolonged bleeding
Give some congenital examples of vascular defects
Osler-weber-rendu syndrome
Connective tissue disease (Ehlers danlos)
Give some acquired examples of vascular defects
Senile purpura Infection - meningococcal, measles, dengue fever Steroids Scurvy (perifollicular haemorrhages) Henoch-schonlein purpura
Give some examples of platelet disorders caused by decreased marrow production
Aplastic anaemia
Megaloblastic anaemia
Marrow infiltration (leukaemia and melanoma)
Marrow suppression (cytotoxic drugs and radiotherapy)
Give some examples of platelet disorders caused by increased destruction
Immune thrombocytopenia (ITP) SLE CLL Drugs - heparin Virus DIC Thrombotic thrombocytopenia (TTP) HUS Sequestration (in hypersplenism)
Give some platelet disorders caused by poorly functioning platelets
Myeloproliferative disease
NSAIDs
Increased urea
Give some examples of congenital coagulation disorders
Haemophilia
Von Willebrand’s disease
Give some examples of acquired coagulation disorders
Anticoagulants
Liver disease
DIC
Vitamin K defiency
What is haemophilia A a deficiency of?
Factor 8
How is haemophilia A inherited?
X-linked recessive condition, however 30% of cases arise from new mutations
Describe the clinical presentation of haemophilia A
Depends on the severity
Often early in life or after surgery/trauma
Bleeds into joints - arthropathy
Bleeding into muscles - haematomas (increased pressure can lead to nerve palsies and compartment syndrome)
How is haemophilia A diagnosed?
Increased APTT
Decreased factor 8 assay
How is haemophilia A managed?
Minor bleeding - pressure and elevation
Major bleeding - desmopressin - raises factor 8 - raised by 50% may be sufficient
Severe bleeding - recombinant factor 8 to raise the levels by 100%
What must be avoided in haemophilia A?
NSAIDs and IM injections
What is haemophilia B otherwise known as?
Christmas disease
How do you treat haemophilia B
Recombinant factor 9