A neutrophil disorder.

Issue is lysosomes, so large vesicles seen in neutrophils which have reduced function. Associated with albinism, developmental delay, bleeding disorder. The connection is that all have granules, and the defect is in way that endoplasmic reticulum sorts different products into different granules.

Griscelli syndrome is similar, associated with albinism but not developmental delay or bleeding. Can predispose to haemophagocytic syndrome. Differentiate from Chediak Higashi by light microscopy of hair (unevenly distributed large melanin granules cf evenly distributed; white under polarized light cf polychromatic).

Hermansky Pudlak II has the albinism, developmental delay and bleeding, but has pigmented macrophages instead of funny neutrophils. The neutrophil numbers as well as function are reduced, whereas other subtypes do not have any immunodeficiency. Check bleeding time.