Tags: Disease.
Hermansky–Pudlak syndrome (HPS) is a rare autosomal recessive disorder which results in oculocutaneous albinism (decreased pigmentation) bleeding problems due to a platelet abnormality (platelet storage pool defect) and storage of an abnormal fat-protein compound (lysosomal accumulation of ceroid lipofuscin).It is considered to affect around 1 in 500000 people worldwide with a significantly higher occurrence in Puerto Ricans with a prevalence of 1 in 1800.