Orphanet rare-disease nomenclature id Orphanet_167 (Chédiak-Higashi syndrome) (CHS) is a rare severe genetic disorder generally characterized by partial oculocutaneous albinism (OCA, see this term), severe immunodeficiency, mild bleeding, neurological dysfunction and lymphoproliferative disorder. A classic, early-onset form and an attenuated, later-onset form (Atypical CHS; see this term) have been described. Also known as: CHS, Chediak - Steinbrinck anomaly, Chediak-Higashi syndrome, Chédiak-Higashi disease, Chédiak-Higashi-Steinbrink syndrome.