Bernard-Soulier Syndrome

Bernard-Soulier syndrome occurs when platelets lack a substance that sticks to the walls of blood vessels. This disorder may cause severe bleeding.

Bernard-Soulier syndrome (giant platelet syndrome): A primary problem of platelets in which the platelets lack the ability to stick adequately to injured blood vessel walls.

What is Bernard-Soulier syndrome?
The Bernard-Soulier Syndrome (B-SS) is a rare inherited bleeding disorder caused by abnormal platelets and subsequent abnormal clotting. It is one of the giant platelet syndromes.

Bernard-Soulier syndrome (BSS) is a rare inherited disorder of blood clotting (coagulation) characterized by unusually large and irregularly shaped platelets; low platelet count (thrombocytopenia) and prolonged bleeding time (difficulty in clotting).

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adhesion (Bernard-Soulier syndrome) Â- aggregation (Glanzmann's thrombasthenia) Â- platelet storage pool deficiency (Hermansky-Pudlak syndrome, Gray platelet syndrome)
Clotting factor ...

Bernard-Soulier Syndrome ... frequent nosebleeds
Bernheim syndrome ... chest pain, shortness of breath, shortness of breath
Bernheim's syndrome ... shortness of breath
Berry aneurysm, cirrhosis, pulmonary emphysema, and cerebral calcification ...

The abnormal platelets in the Bernard-Soulier syndrome are usually considerably larger than normal platelets when viewed on blood films or sized by automated instruments. However, this is not the only syndrome with large platelets.

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See also: Bleeding, Symptom, Deficiency, Bleeding disorder, Cancer