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. 1973 Mar;70(3):669–672. doi: 10.1073/pnas.70.3.669

Congenital Neutropenia: In Vitro Growth of Colonies Mimicking the Disease

Pierre L'Esperance *, Richard Brunning †, Robert A Good *
PMCID: PMC433331  PMID: 4514979

Abstract

Congenital neutropenia is a lethal disease characterized by recurrent infections beginning in the neonatal period, absence of neutrophils in the peripheral blood, eosinophilia, and monocytosis. The bone marrow shows an apparent “maturation arrest” of the neutrophil series at the promyelocyte stage. Granulocytic colonies grown in vitro in soft agar medium show normal development of eosinophilic colonies and monocyte-macrophage colonies, but defective neutrophil maturation. The abnormal colonies observed contained only myeloblasts and promyelocytes. Thus, it seems to have been possible to mimic in vitro the abnormal differentiation that is observed in vivo.

Keywords: hematopoietic, defective stem cell, abnormal clone, differentiation

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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