A patient sample has normal platelet aggregation patterns for all agonists except that no reaction occurs with ristocetin. Ristocetin agglutination is not corrected with exogenous VWF. What condition is most likely?
a. VWD
b. Storage pool disorder
c. Bernard-Soulier syndrome
d. Aspirin use
ANS: C
Platelets from patients with Bernard-Soulier syndrome do not agglutinate with ristocetin, and it is not corrected when exogenous VWF is added. Their platelets have an intrinsic membrane defect, a deficiency of the GP1b or IX portion of the GP1b/V/IX receptor. This is in contrast to platelets from patients with VWD; they also do not agglutinate with ristocetin, but agglutination is corrected when exogenous VWF is added. Platelets from patients with storage pool disorder or those taking aspirin agglutinate normally with ristocetin.
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