INFANTILE GENETIC AGRANULOCYTOSIS ASSOCIATED WITH CHANGES IN SERUM VITAMIN B<sub>12</sub> BINDING PROTEINS

Beard, Michael; Newmark, Peter; Smith, M E Beth; Franklin, A.

doi:10.1111/j.1651-2227.1972.tb15939.x

Acta Paediatrica

1972

DOI: 10.1111/j.1651-2227.1972.tb15939.x

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INFANTILE GENETIC AGRANULOCYTOSIS ASSOCIATED WITH CHANGES IN SERUM VITAMIN B₁₂ BINDING PROTEINS

Michael Beard¹,

Peter Newmark²,

M E Beth Smith³

et al.

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Infantile Genetic Agranulocytosis

Kostman¹

1975

Acta Paediatrica

253

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A review of the literature on the subject since 1956 is made in connection with a presentation of ten new cases from northern Sweden. Nine of these are related to the main pedigree published in 1956. Consanguinity between the parents has been established in two of the new families. The clinical course was identical to that described in 1956. A few additional details are presented. The granulocytopenia is present on the first day of life and the granulocyte count subsequently rapidly decreases during the first week. The existence of a diaplacental factor is regarded highly probable. It is assumed that the maturation defect in the granulocyte precursors may be due to deficiency of a serum factor. The fact that many cases of infantile genetic agranulocytosis occur sporadically is finally explained.

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Infantile Genetic Agranulocytosis

Kostman¹

1975

Acta Paediatrica

253

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Chronic idiopathic neutropenia with hypergammaglobulinemia.

et al. 1987

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Chronic idiopathic neutropenia can occur in spite of the normocellular bone marrowin myeloid series with or without anti-neutrophilic antibody. We report a patient with chronic neutropenia and severe polyclonal hypergammaglobulinemia.The patient demonstrated a positive anti-neutrophil antibody by fluorocytometry, although granulocyte-specific anti-nuclear factor and anti-stem cell (CFU-GM) antibody were negative. Thus, neutropenia of this patient appeared to be due to the antibody-mediated destruction of neutrophils. Both neutropenia and hypergammaglobulinemia were normalized by the administration of prednisone.

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INFANTILE GENETIC AGRANULOCYTOSIS ASSOCIATED WITH CHANGES IN SERUM VITAMIN B₁₂ BINDING PROTEINS

Cited by 2 publications

References 24 publications

Infantile Genetic Agranulocytosis

Infantile Genetic Agranulocytosis

Chronic idiopathic neutropenia with hypergammaglobulinemia.

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INFANTILE GENETIC AGRANULOCYTOSIS ASSOCIATED WITH CHANGES IN SERUM VITAMIN B12 BINDING PROTEINS

Cited by 2 publications

References 24 publications

Infantile Genetic Agranulocytosis

Infantile Genetic Agranulocytosis

Chronic idiopathic neutropenia with hypergammaglobulinemia.

Contact Info

Product

Resources

About

INFANTILE GENETIC AGRANULOCYTOSIS ASSOCIATED WITH CHANGES IN SERUM VITAMIN B₁₂ BINDING PROTEINS