PEDIATRICS Vol. 35 No. 4 April 1965, pp. 596-600
This Article
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Lang, J. E.
Right arrow Articles by Cutting, H. O.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Lang, J. E.
Right arrow Articles by Cutting, H. O.

INFANTILE GENETIC AGRANULOCYTOSIS

J. Eugene Lang M.D., USN (M.C.)1 and Hunter O. Cutting M.D., LCDR., USNR (M.C.)2

1 Department of Hematology, U.S. Naval Hospital, San Diego, California
2 Department of Internal Medicine, U.S. Naval Hospital, San Diego, California

A 4frac12-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.

Submitted on August 12, 1964
Accepted on November 4, 1964