Defective platelet autocrine signaling in HPS

Abstract

In this issue of Blood, Meng et al1 and Sharda et al2 use the Hermansky-Pudlak syndrome (HPS) as a model to show that adenosine 5'-diphosphate (ADP) released by dense granules serves as an autocrine signal to potentiate platelet release of α-granule and lysosome cargo and protein disulfide isomerase (PDI), all of which serve to stabilize thrombus formation.