INFANTILE GENETIC AGRANULOCYTOSIS

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 primar...

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Bibliographic Details
Published in:Pediatrics (Evanston) 1965-04, Vol.35 (4), p.596-600
Main Authors: LANG, J E, CUTTING, H O
Format: Article
Language:English
Subjects:
Agranulocytosis
Bone Marrow Examination
Child
Diagnosis
Eosinophils
Genetics, Medical
Humans
Leukocyte Count
Lymphocytes
Monocytes
Neutropenia
Neutrophils
Old Medline
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Summary: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.
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.35.4.596