The results of platelet-rich plasma (PRP) in the aggregation studies of a patient's blood were typical of ristocetin-induced platelet agglutination (RIPA) defect. The RIPA defect was corrected by adding normal plasma to the patient's PRP. In what disease is this seen?
A) Sticky platelet syndrome
B) Bernard-Soulier syndrome
C) Afibrinogenemia
D) von Willebrand's disease

Question

Quizplus · Accepted Answer

The platelet aggregation defect seen in the patient is consistent with RIPA defect, which is a characteristic finding in von Willebrand's disease. Adding normal plasma to the patient's PRP corrected the defect, which indicates a deficiency or dysfunction of vWF in the patient's plasma. Sticky platelet syndrome is a rare disorder characterized by platelet hyperaggregability, but it is not associated with RIPA defect. Bernard-Soulier syndrome is associated with a defect in the glycoprotein Ib-IX-V receptor, and afibrinogenemia is a rare bleeding disorder characterized by the absence of fibrinogen. Neither of these disorders is associated with RIPA defect.

The Results of Platelet-Rich Plasma (PRP) in the Aggregation Studies