Effects of normal and aspirin platelets on defective secondary aggregation in the Hermansky-Pudlak syndrome. A test for storage pool deficient platelets.

Abstract

Platelets from patients with the Hermansky-Pudlak syndrome (albinism, accumulation of ceroid-like pigment in bone marrow macrophages and mild hemorrhagic symptoms) and aspirin-treated normal platelets fail to develop irreversible secondary waves of aggregation when exposed to aggregating agents on the platelet aggregometer. The present study demonstrates that equal volumes of Hermansky-Pudlak and aspirin-treated platelets, when mixed together, respond in a biphasic manner comparable to normal cells. Correction of the release defect of aspirin platelets by storage pool deficient Hermansky-Pudlak cells indicates that different mechanisms are involved in the failure of the release reaction in the two cell types. The simple test may serve to distinguish other patients with storage pool deficient platelets from individuals who have taken aspirin or other drugs with an aspirin-like effect on platelet function.