Congenital Neutropenia

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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

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