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Blood mononuclear cells from patients with severe congenital neutropenia
are capable of producing granulocyte colony-stimulating factor
T Pietsch, C Buhrer, K Mempel, T Menzel, U Steffens, C Schrader, F Santos, C Zeidler and K Welte
Department of Pediatric Hematology and Oncology, Children's Hospital,
Hannover Medical School, Germany.
Severe congenital neutropenia (SCN) is a disorder of myelopoiesis
characterized by severe neutropenia or absence of blood neutrophils
secondary to a maturational arrest at the level of promyelocytes. We
examined peripheral blood mononuclear cells (PBMC) of SCN patients who
demonstrated normalization of their blood neutrophil counts in a phase II
clinical study with recombinant human granulocyte colony-stimulating factor
(rhG-CSF). When stimulated in vitro with bacterial lipopolysaccharides
(LPS), PBMC of those SCN patients produced G-CSF activity, as judged by
proliferation induction of the murine leukemia cell line, NFS-60. Western
and Northern blot analysis showed G-CSF protein and G-CSF-mRNA
indistinguishable in size from those of normal controls. We conclude that
PBMC of the SCN patients tested are capable of synthesizing and secreting
biologically active G-CSF in vitro.
Volume 77,
Issue 6,
pp. 1234-1237,
03/15/1991
Copyright © 1991 by The American Society of Hematology
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