Hematopathology — Image #22 — Bernard Soulier Syndrome

Bernard Soulier Syndrome

This inherited disorder is characterized by decreased numbers of platelets, which are also defective, leading to severe bleeding. The platelet membrane is abnormal; the platelets are "large and floppy." Most significantly, the lack GP Ib-IX, and therefore cannot interact with von Willebrand's factor to attach to exposed subendothelium. The enormous pink platelet in the image is from a patient with Bernard Soulier Syndrome.

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