Hermansky-Pudlak syndrome (HPS5) in a nonagenarian.

[oculocutaneous albinism]

Hermansky-Pudlak syndrome (HPS ) is an autosomal-recessive disorder clinically characterized by oculocutaneous albinism , bleeding diatheses , and lysosomal accumulation of ceroid lipofuscin , which in some cases may cause granulomatous colitis and pulmonary fibrosis . Any of these complications could result in a shortened life span for patients with HPS . We report a 92 -year -old man with HPS 5 who , to our knowledge , is the oldest patient with HPS documented in the literature . This report highlights the importance of typing HPS to counsel patients regarding disease prognosis .