A 4½-year-old white boy has been followed for 14 months with chronic agranulocytosis. This case is an example of the rare, non-sex linked recessive disease of the hematopoietic system, first described definitely by Kostman in 1956, and termed "Infantile Genetic Agranulocytosis." The primary defect is a severe depression of the neutrophilic granulocytes. The other marked morphological and clinical abnormalities we believe are secondary to the neutropenia and the attending infections. A brief comparison with other "primary" chronic neutropenic states reported in childhood and clinically similar to infantile genetic agranulocytosis has been made. The differential diagnostic features are stressed.
- Received August 12, 1964.
- Accepted November 4, 1964.
- Copyright © 1965 by the American Academy of Pediatrics