What is the characteristic histological finding in Bernard-Soulier Disease?

In Bernard-Soulier Disease, the characteristic histological finding is the presence of large platelets. This condition is a rare inherited bleeding disorder caused by a deficiency of the glycoprotein complex on the platelet membrane, specifically glycoprotein Ib-IX-V, which is crucial for the platelets' ability to bind to von Willebrand factor (vWF).

The large platelets arise as a compensatory mechanism; the bone marrow increases platelet production in response to the ineffective platelet function and reduced platelet survival, leading to large, immature platelets being released into the circulation. These platelets are often referred to as "giant platelets" and can be readily recognized in a peripheral blood smear.

Understanding the pathophysiology of the disease and recognizing the histological features are important for diagnosing Bernard-Soulier Disease. The appearance of these large platelets is a distinguishing feature that can aid in differentiating this condition from other platelet disorders. Other choices, while related to platelet abnormalities, do not specifically characterize Bernard-Soulier Disease as effectively as the large platelets do.