Golgi complexes in hypogranular platelet syndromes

The white platelet syndrome (WPS) is an autosomal dominantly inherited hypogranular platelet disorder characterized by the presence of fully developed Golgi complexes from parent megakaryocytes in 13% or more of their circulating platelets. The present study has evaluated several other hypogranular platelet disorders to determine if perpetuation of Golgi complexes in circulating cells is a common link in those inherited conditions. Only platelets from patients with the gray platelet syndrome (GPS) and one patient with alpha delta platelet storage pool deficiency (alpha delta SPD) had more Golgi complexes in their cells than were found in normal thrombocytes. Platelets from patients with the Hermansky-Pudlak syndrome, two other patients with alpha delta SPD and the individual with Medich giant platelet disorder had the same frequency of Golgi complexes in circulating cells as normal controls. Thus, the presence of large numbers of fully developed Golgi complexes in circulating platelets appears unique to the WPS.