There are over 25,000 copies of GPIb-complex on the surface of an average normal platelet. In BSS, there are no copies of GPIb-complex. This does not make clotting impossible, it means that platelets must be stimulated by, for example, adrenaline, before they will clot normally. Symptoms Symptoms include a combination of the following. Enlarged, abnormal platelets.
Treatment usually involves infusion of platelet activating factors, and platelet transfusion.
For which a common problem is development of allantibodies to the infused platelets. Vasopressin is a stimulaten of platelets. IN Scriver C.R., Beaudet A.L., William S.S., editors, Molecular and Metabolic basis of disease.