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Infantile Genetic Agranulocytosis: Title and subTitle BreakTwo Cases Occurring in Siblings and One in a Distant Relative FREE

Alvin E. Rodin, MD; Mary E. Haggard, MD; Myron M. Nichols, MD; Lillian P. Gustavson, MD
[+] Author Affiliations

Received for publication Oct 19, 1972; accepted July 12, 1973.

Reprint requests to Department of Pathology, University of Texas Medical Branch, Galveston TX 77550 (Dr. Rodin).


Arch Pediatr Adolesc Med. 1973;126(6):818-821. doi:10.1001/archpedi.1973.02110190660016
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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.

REFERENCES

Wintrobe MM: Clinical Hematology . Philadelphia, Lea & Febiger, 1967;, pp 238, 795-796, and 1221-1222.
Kostman R:  Infantile genetic agranulocytosis (agranulocytosis infantilis hereditaria): A new recessive lethal disease of man . Acta Paediatr 45( (suppl 105) ):1-79, 1956;.
Kniker WT, Panos TC:  Idiopathic infantile agranulocytosis with hypergammaglobulinemia . AMA J Dis Child 94:549-550, 1957;.
Luhby AL, et al:  Congenital genetic agranulocytosis . AMA J Dis Child 94:552-553, 1957;.
Hedenberg F:  Infantile agranulocytosis of probably congenital origin . Acta Paediatr 48:77-84, 1959;.
Andrews JP, McClellan JT, Scott CH:  Lethal congenital neutropenia with eosinophilia occurring in two siblings . Am J Med 29:358-362, 1960;.
Aarskog D:  Infantile congenital aneutrocytosis . Arch Dis Child 36:511-514, 1961;.
MacGillivray JB, et al:  Congenital neutropenia: A report of five cases . Acta Paediatr 53:188-203, 1964;.
Lang JE, Cutting HO:  Infantile genetic agranulocytosis . Pediatrics 35:596-600, 1965;.
Krill CE, Mauer AM:  Congenital agranulocytosis . J Pediatr 68:361-366, 1966;.
Reading RS: Arrows Over Texas . San Antonio, Tex, Naylor Co, 1960;, pp 209-212.
Schneider RG, et al:  Hemoglobin Gcoushatta: A new variant in an American Indian family . Science 143:697-698, 1964;.

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Wintrobe MM: Clinical Hematology . Philadelphia, Lea & Febiger, 1967;, pp 238, 795-796, and 1221-1222.
Kostman R:  Infantile genetic agranulocytosis (agranulocytosis infantilis hereditaria): A new recessive lethal disease of man . Acta Paediatr 45( (suppl 105) ):1-79, 1956;.
Kniker WT, Panos TC:  Idiopathic infantile agranulocytosis with hypergammaglobulinemia . AMA J Dis Child 94:549-550, 1957;.
Luhby AL, et al:  Congenital genetic agranulocytosis . AMA J Dis Child 94:552-553, 1957;.
Hedenberg F:  Infantile agranulocytosis of probably congenital origin . Acta Paediatr 48:77-84, 1959;.
Andrews JP, McClellan JT, Scott CH:  Lethal congenital neutropenia with eosinophilia occurring in two siblings . Am J Med 29:358-362, 1960;.
Aarskog D:  Infantile congenital aneutrocytosis . Arch Dis Child 36:511-514, 1961;.
MacGillivray JB, et al:  Congenital neutropenia: A report of five cases . Acta Paediatr 53:188-203, 1964;.
Lang JE, Cutting HO:  Infantile genetic agranulocytosis . Pediatrics 35:596-600, 1965;.
Krill CE, Mauer AM:  Congenital agranulocytosis . J Pediatr 68:361-366, 1966;.
Reading RS: Arrows Over Texas . San Antonio, Tex, Naylor Co, 1960;, pp 209-212.
Schneider RG, et al:  Hemoglobin Gcoushatta: A new variant in an American Indian family . Science 143:697-698, 1964;.

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