[Skip to Navigation]
December 1973

Infantile Genetic Agranulocytosis: Two Cases Occurring in Siblings and One in a Distant Relative

Author Affiliations

Galveston, Tex
From the departments of pathology (Dr. Rodin) and pediatrics (Drs. Haggard, Nichols, and Gustavson), University of Texas Medical Branch, Galveston.

Am J Dis Child. 1973;126(6):818-821. doi:10.1001/archpedi.1973.02110190660016

Infantile genetic agranulocytosis (IGA) is characterized by agranulocytosis with variable monocytosis in the peripheral blood, vacuolated "monocytoid" cells and decreased mature neutrophils in the marrow, repeated infections with death in infancy, and inflammatory lesions with necrotic centers but no neutrophilic response. Three infants of an east Texas Indian tribe, two siblings and one with a common paternal greatgrandfather, are now reported. In addition to the characteristic features, all three had mental retardation, and two had congenital cataracts, providing some support for a genetic basis for IGA.