Which statement is true regarding Bernard-Soulier syndrome and von Willebrand disease?

Which statement is true regarding Bernard-Soulier syndrome and von Willebrand disease?

• A. Giant platelets are a feature of Bernard-Soulier but not typically of von Willebrand disease
• B. Giant platelets are a feature of von Willebrand disease but not of Bernard-Soulier
• C. Both conditions always show giant platelets on smear
• D. Neither condition shows giant platelets

Answer: (A)

Giant platelets on smear are a classic feature of Bernard-Soulier syndrome, which is caused by a defect in the GPIb-IX-V complex that impairs platelet adhesion to von Willebrand factor. This abnormality also leads to thrombocytopenia and larger-than-normal platelets. In von Willebrand disease, the primary problem is deficient or defective von Willebrand factor, leading to impaired platelet adhesion, but the platelets themselves are typically normal in size. Therefore, giant platelets are characteristic of Bernard-Soulier but not of von Willebrand disease. The other statements are inconsistent with how each condition affects platelet size.

Giant platelets on smear are a classic feature of Bernard-Soulier syndrome, which is caused by a defect in the GPIb-IX-V complex that impairs platelet adhesion to von Willebrand factor. This abnormality also leads to thrombocytopenia and larger-than-normal platelets. In von Willebrand disease, the primary problem is deficient or defective von Willebrand factor, leading to impaired platelet adhesion, but the platelets themselves are typically normal in size. Therefore, giant platelets are characteristic of Bernard-Soulier but not of von Willebrand disease. The other statements are inconsistent with how each condition affects platelet size.