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Congenital Neutropenia: Report of a Fatal Case in a Negro Infant With Leukocyte Function Studies

Congenital Neutropenia: Report of a Fatal Case in a Negro Infant With Leukocyte Function Studies Abstract CONGENITAL neutropenia is a rare disease characterized by markedly decreased or absent peripheral blood neutrophils and totally depressed granulopoiesis beyond the promyelocyte-myelocyte stage. Erythropoiesis and megakaryocytes are normal. Eosinophilia, monocytosis, hypergammaglobulinemia, and recurrent infections are associated findings and death in early infancy is the usual outcome. Diffuse inflammatory reactions devoid of neutrophils and consisting predominantly of lymphocytes, plasma cells, and histiocytes are seen in postmortem tissues. Although the classic family studies of Kostmann1 and the siblings described by Andrews et al2 suggest an autosomal recessive mode of inheritance, other single fetal cases3-8 have not demonstrated a familial predisposition. The first report of this syndrome in a Negro infant and the results of leukocyte function studies form the basis of this paper. Report of a Case A Negro girl (URMC-SMH 631912) was the product of a 30-year-old gravida 7, para 5, abortio 1 mother who was Rh positive References 1. Kostmann, R.: Infantile Genetic Agranulocytosis , Acta Paediat 45 ( (suppl 105) ):1, 1956.Crossref 2. Andrews, J.P.; McClellan, J.T.; and Scott, C.H.: Lethal Congenital Neutropenia With Eosinophilia Occurring in Two Siblings , Amer J Med 29: 358, 1960.Crossref 3. Kniker, W.T., and Panos, T.C.: Idiopathic Infantile Agranulocytosis With Hypergammaglobulinemia , abstract, Amer J Dis Child 94:549, 1957. 4. Luhby, A.L., et al: Congenital Genetic Agranulocytosis , abstract, Amer J Dis Child 94: 552, 1957. 5. Hedenberg, F.: Infantile Agranulocytosis of Probably Congenital Origin , Acta Paediat 48:77, 1959. 6. Aarskog, D.: Infantile Congenital Aneutrocytosis , Arch Dis Child 36:511, 1961.Crossref 7. MacGillivray, J.B., et al: Congenital Neutropenia: A Report of Five Cases , Acta Paediat 53: 188, 1964.Crossref 8. Krill, C.E., and Mauer, A.M.: Congenital Agranulocytosis , J Pediat 68:361, 1966.Crossref 9. Vaughan, J.H.; Jacox, R.F.; and Gray, B.A.: Light and Heavy Chain Components of Gamma Globulins in Urine of Normal Persons and Patient With Agammaglobulinemia , J Clin Invest 46:266, 1967.Crossref 10. Rebuck, J.W., and Crowley, J.H.: Method of Studying Leukocyte Functions in vivo , Ann New York Acad Sci 59:757, 1955.Crossref 11. Lalezari, P., et al: Neonatal Neutropenia Due to Maternal Isoimmunization , Blood 15:236, 1960. 12. Good, R.A., and Zok, S.J.: Disturbances in Gammaglobulin Synthesis as "Experiments of Nature," Pediatrics 18:109, 1956. 13. Hong, R., et al: Antibody Deficiency Syndrome With β-2 Macroglobulinemia , J Pediat 61: 831, 1962.Crossref 14. Shwachman, H., et al: The Syndrome of Pancreatic Insufficiency and Bone Marrow Dysfunction , J Pediat 65:645, 1964.Crossref 15. Childs, B., et al: Idiopathic Hyperglycinemia and Hyperglycinuria: A New Disorder of Amino Acid Metabolism , Pediatrics 27:522, 1961. 16. Van Roud, J.J.; van Leeuwen, A.; and Cernisse, J.G.: Leukocyte Antibodies in Pregnant Women , Vox Sang 4:427, 1959.Crossref 17. Jensen, K.G.: Leukocyte Antibodies in Serums of Pregnant Women , Vox Sang 7:454, 1962.Crossref 18. Payne, R.: Neonatal Neutropenia and Leukoagglutinins , Pediatrics 33:194, 1964. 19. Zeulzer, W.C., and Bajoghli, M.: Chronic Granulocytopenia in Childhood , Blood 23:359, 1964. 20. Krill, C.E., Jr.; Smith, H.D.; and Mauer, A.M.: Chronic Idiopathic Granulocytopenia , New Eng J Med 270:973, 1964.Crossref 21. Jobler, W., and Buser-Pluess, E.: Beobachtungen in einem Falle von chronischer myelogener, hochgradiger Neutropenie mit monocytaerer Reaktion , Ann Paediat 156:258, 1942. 22. Wriedt, K.; Kauder, E.; and Mauer, A.M.: Failure of Myeloid Differentiation as a Cause of Congenital Neutropenia , abstract, J Pediat 68:839, 1966. 23. Bjure, J.; Nilsson, L.R.; and Plum, C.M.: Familial Neutropenia Possibly Caused by Deficiency of a Plasma Factor , Acta Paediat 51:497, 1962.Crossref 24. Brown, G.O., Jr.; Herbig, F.K.; and Hamilton, J.R.: Leukopenia in Negroes , New Eng J Med 275: 1410, 1966.Crossref 25. Gairdner, D.; Marks, J.; and Roscoe, J.D.: Blood Formation in Infancy: I. The Normal Bone Marrow , Arch Dis Child 27:128, 1952.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Congenital Neutropenia: Report of a Fatal Case in a Negro Infant With Leukocyte Function Studies

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References (29)

Publisher
American Medical Association
Copyright
Copyright © 1968 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1968.02100010339007
Publisher site
See Article on Publisher Site

Abstract

Abstract CONGENITAL neutropenia is a rare disease characterized by markedly decreased or absent peripheral blood neutrophils and totally depressed granulopoiesis beyond the promyelocyte-myelocyte stage. Erythropoiesis and megakaryocytes are normal. Eosinophilia, monocytosis, hypergammaglobulinemia, and recurrent infections are associated findings and death in early infancy is the usual outcome. Diffuse inflammatory reactions devoid of neutrophils and consisting predominantly of lymphocytes, plasma cells, and histiocytes are seen in postmortem tissues. Although the classic family studies of Kostmann1 and the siblings described by Andrews et al2 suggest an autosomal recessive mode of inheritance, other single fetal cases3-8 have not demonstrated a familial predisposition. The first report of this syndrome in a Negro infant and the results of leukocyte function studies form the basis of this paper. Report of a Case A Negro girl (URMC-SMH 631912) was the product of a 30-year-old gravida 7, para 5, abortio 1 mother who was Rh positive References 1. Kostmann, R.: Infantile Genetic Agranulocytosis , Acta Paediat 45 ( (suppl 105) ):1, 1956.Crossref 2. Andrews, J.P.; McClellan, J.T.; and Scott, C.H.: Lethal Congenital Neutropenia With Eosinophilia Occurring in Two Siblings , Amer J Med 29: 358, 1960.Crossref 3. Kniker, W.T., and Panos, T.C.: Idiopathic Infantile Agranulocytosis With Hypergammaglobulinemia , abstract, Amer J Dis Child 94:549, 1957. 4. Luhby, A.L., et al: Congenital Genetic Agranulocytosis , abstract, Amer J Dis Child 94: 552, 1957. 5. Hedenberg, F.: Infantile Agranulocytosis of Probably Congenital Origin , Acta Paediat 48:77, 1959. 6. Aarskog, D.: Infantile Congenital Aneutrocytosis , Arch Dis Child 36:511, 1961.Crossref 7. MacGillivray, J.B., et al: Congenital Neutropenia: A Report of Five Cases , Acta Paediat 53: 188, 1964.Crossref 8. Krill, C.E., and Mauer, A.M.: Congenital Agranulocytosis , J Pediat 68:361, 1966.Crossref 9. Vaughan, J.H.; Jacox, R.F.; and Gray, B.A.: Light and Heavy Chain Components of Gamma Globulins in Urine of Normal Persons and Patient With Agammaglobulinemia , J Clin Invest 46:266, 1967.Crossref 10. Rebuck, J.W., and Crowley, J.H.: Method of Studying Leukocyte Functions in vivo , Ann New York Acad Sci 59:757, 1955.Crossref 11. Lalezari, P., et al: Neonatal Neutropenia Due to Maternal Isoimmunization , Blood 15:236, 1960. 12. Good, R.A., and Zok, S.J.: Disturbances in Gammaglobulin Synthesis as "Experiments of Nature," Pediatrics 18:109, 1956. 13. Hong, R., et al: Antibody Deficiency Syndrome With β-2 Macroglobulinemia , J Pediat 61: 831, 1962.Crossref 14. Shwachman, H., et al: The Syndrome of Pancreatic Insufficiency and Bone Marrow Dysfunction , J Pediat 65:645, 1964.Crossref 15. Childs, B., et al: Idiopathic Hyperglycinemia and Hyperglycinuria: A New Disorder of Amino Acid Metabolism , Pediatrics 27:522, 1961. 16. Van Roud, J.J.; van Leeuwen, A.; and Cernisse, J.G.: Leukocyte Antibodies in Pregnant Women , Vox Sang 4:427, 1959.Crossref 17. Jensen, K.G.: Leukocyte Antibodies in Serums of Pregnant Women , Vox Sang 7:454, 1962.Crossref 18. Payne, R.: Neonatal Neutropenia and Leukoagglutinins , Pediatrics 33:194, 1964. 19. Zeulzer, W.C., and Bajoghli, M.: Chronic Granulocytopenia in Childhood , Blood 23:359, 1964. 20. Krill, C.E., Jr.; Smith, H.D.; and Mauer, A.M.: Chronic Idiopathic Granulocytopenia , New Eng J Med 270:973, 1964.Crossref 21. Jobler, W., and Buser-Pluess, E.: Beobachtungen in einem Falle von chronischer myelogener, hochgradiger Neutropenie mit monocytaerer Reaktion , Ann Paediat 156:258, 1942. 22. Wriedt, K.; Kauder, E.; and Mauer, A.M.: Failure of Myeloid Differentiation as a Cause of Congenital Neutropenia , abstract, J Pediat 68:839, 1966. 23. Bjure, J.; Nilsson, L.R.; and Plum, C.M.: Familial Neutropenia Possibly Caused by Deficiency of a Plasma Factor , Acta Paediat 51:497, 1962.Crossref 24. Brown, G.O., Jr.; Herbig, F.K.; and Hamilton, J.R.: Leukopenia in Negroes , New Eng J Med 275: 1410, 1966.Crossref 25. Gairdner, D.; Marks, J.; and Roscoe, J.D.: Blood Formation in Infancy: I. The Normal Bone Marrow , Arch Dis Child 27:128, 1952.Crossref

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Mar 1, 1968

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