Megakaryocytes (arrows) In terms of diagnosis<unk>  is characterized by prolonged bleeding time, thrombocytopenia, increased megakaryocytes, and enlarged platelets,<unk>  is associated with quantitative or qualitative defects of the platelet glycoprotein complex GPIb/V/IX. The degree of thrombocytopenia may be estimated incorrectly, due to the possibility that when the platelet count is performed with automatic counters, giant platelets may reach the size of red blood cells. The large platelets and low platelet count in BSS are seemingly due to the absence of GPIbα and the filamin A binding site that links the GPIb-IX-V complex to the platelet membrane skeleton. Differential diagnosis The differential diagnosis for<unk>  includes both Glanzmann thrombasthenia and pediatric Von Willebrand disease. BSS platelets do not aggregate to ristocetin, and this defect is not corrected by the addition of normal plasma, distinguishing it from von Willebrand disease. Following is a table comparing its result with other platelet aggregation disorders:
Bernard–Soulier syndrome
Fryns-Aftimos syndrome
Harris platelet syndrome
Ehlers–Danlos syndromes
Quebec platelet disorder
Keutel syndrome
Von Willebrand disease
Flammer syndrome