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Physiology · Blood and immunity: platelet adhesion
Sample itemAn infant has thrombocytopenia with giant platelets and mucosal bleeding. Ristocetin does not agglutinate the platelets, and adding normal plasma does not correct this. Which defect fits best?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Absent platelet glycoprotein Ib-IX-V receptor
Adhesion to subendothelium needs platelet GPIb to bind von Willebrand factor. When the receptor is missing, as in Bernard-Soulier syndrome, ristocetin agglutination fails and normal plasma cannot correct it because vWF itself is normal. Giant platelets and low counts are typical.
Option by option
A. Absent glycoprotein IIb/IIIa
Absent GPIIb/IIIa gives normal ristocetin agglutination and normal platelet size.
B. Absent platelet glycoprotein Ib-IX-V receptor (correct)
Correct. The platelet receptor for vWF is missing, so adding normal plasma does not help.
C. Deficient factor VIII
Factor VIII deficiency prolongs the aPTT but does not impair ristocetin agglutination.
D. Deficient plasma von Willebrand factor
Plasma vWF deficiency would be corrected by adding normal plasma and platelets would be of normal size.
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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