Chediak-Higashi Syndrome

**Definition:** 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.

**Tree numbers:** - C11.270.040.772 - C15.378.553.774.257 - C16.320.798.375 - C20.673.774.257 - C20.673.795.375

**Synonyms:** - Chediak-Steinbrinck-Higashi Syndrome - Oculocutaneous Albinism with Leukocyte Defect