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Physiology · Blood and immunity: Glanzmann thrombasthenia
Sample itemA child has easy mucosal bleeding, a normal platelet count and a prolonged bleeding time. Platelets fail to aggregate with ADP, collagen and thrombin but agglutinate normally with ristocetin. Which platelet defect fits best?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Deficient glycoprotein IIb/IIIa
In Glanzmann thrombasthenia GPIIb/IIIa, the fibrinogen receptor, is deficient, so platelets cannot aggregate whatever the agonist. Ristocetin agglutination depends on GPIb and von Willebrand factor, which are intact, so it is normal.
Option by option
A. Deficient glycoprotein IIb/IIIa (correct)
Correct. Absent GPIIb/IIIa abolishes aggregation but leaves ristocetin agglutination intact.
B. Deficient dense granule ADP storage
Storage pool defect impairs the second wave of aggregation but not primary aggregation to all agonists.
C. Deficient von Willebrand factor
Von Willebrand deficiency would also show abnormal ristocetin response and a prolonged aPTT.
D. Deficient glycoprotein Ib-IX
Absent GPIb-IX (Bernard-Soulier) would give abnormal ristocetin agglutination and large platelets.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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