In 1952 Dr. Chediak reported a childhood disorder in which abnormal cytoplasmic inclusions appeared in the neutrophils of four family members. In 1954 Higashi reported a similar abnormality in an 11-month old Japanese infant. These inclusions were identified as lysosomal in origin and found in this rare autosomal recessive disorder.
Death in patients afflicted with this condition was usually related to recurrent infections or hemorrhage. Ocular and cutaneous albinism, increased susceptibility to pyogenic infections, abnormal granules in neutrophils, and a bleeding tendency are all prominent findings in Chediak-Higashi anomaly.
Notice the striking neutrophilic inclusions which appear as coarse intra-cytoplasmic azurophilic granules (indicated by the arrows in the images on the right). These granules arise from dilated portions of the Golgi-endoplasmic reticulum lysosomal apparatus.
