Glanzmann’s thrombasthenia- AR
GPIIb/IIIa defect - faulty platelet aGGregation + diminished clot retraction.
• @ Birth – neonatal purpura, mucocutaneous bleeding and spontaneous bruising.
• Mostly diagnosed <5years – Epistaxis, minor trauma + bleeding/ bruises
• @ puberty in females- menorrhagia + IDA
• PPH in peripartum period
• H/o bleeding during hemostatic challenges
• Normal PLT Count/morphology
• Normal Coagulation
• BT Deranged
• LTA -Diagnostic- Plts fail to aggregate with all agonist except ristocetin.
• PFA- Prolonged
• Usually stable until hemostatic challenge occurs – dental extraction, trauma, surgery, menarche .
• Avoid contact sports, NSAIDS and anti inf drugs
• Hep B immunisation – I/V/O repeated Plt Tx possibility.
• HLA typing and HLA matched plts ideal to avoid alloimmunisation and refractoriness in future (Abs also known to develop against 2b/3a
• I/C/O Bleeding episodes – local measures + tranexamic acid (CI in hematuria)
• PLT Tx- failure of std measures/ Sx prophylaxis if needed should be preferably HLA matched Single donor platelets to reduce exposure
• RVIIa- useful by unknown mechanism, important in alloimmunised patients
• DDAVP- no role
Mnemonic – Bernard soulier – 1B – Gp 1b/IX
Gl A N zm A NN – 2A/3N= Gp 2B/3A