Testing for Bernard-Soulier Syndrome
In some people with BSS, the platelet count is usually mild to moderately low. Some individuals may have very low platelet counts, while others have normal counts.
A person with BSS may take more than 20 minutes for bleeding to stop from a small cut. Their results with the PFA 100® are expected to be abnormal.
When viewed under the microscope, the platelets of people with BSS are much larger than normal.
An important test for BSS is platelet aggregation. When the antibiotic ristocetin is mixed with the individual’s platelets, it does not make them clump together as it would with normal platelets.