A form of phagocyte bactericidal dysfunction characterized by unusual oculocutaneous albinism, high incidence of lymphoreticular neoplasms, and recurrent pyogenic infections. In many cell types, abnormal lysosomes are present leading to defective pigment distribution and abnormal neutrophil functions. The disease is transmitted by autosomal recessive inheritance and a similar disorder occurs in the beige mouse, the Aleutian mink, and albino Hereford cattle.

DUI

D002609 MeSH Browser

CUI

M0003997

Previous indexing

Immunologic Deficiency Syndromes (1968-1970); Leukocytes (1966-1970)

History note

71

Public note

71