|
|
 Previous Article | Table of Contents | Next Article 
Abnormal responses of myeloid progenitor cells to recombinant human
colony-stimulating factors in congenital neutropenia
M Kobayashi, C Yumiba, Y Kawaguchi, Y Tanaka, K Ueda, Y Komazawa and K Okada
Department of Pediatrics, Hiroshima University School of Medicine, Japan.
The effects of recombinant human interleukin-3 (IL-3) and recombinant human
granulocyte colony-stimulating factor (G-CSF) on the growth of myeloid
progenitor cells (CFU-C) in semisolid agar culture were studied in two
patients with Kostmann-type congenital neutropenia. CFU-C growth in bone
marrow cells from patients was significantly reduced in response to various
concentrations of either IL-3 or G-CSF alone, compared with that from
normal subjects. There was no inhibitory effect of bone marrow cells from
patients on normal CFU-C formation supported by IL-3 or G-CSF. However, the
simultaneous stimulation with IL-3 and G- CSF induced the increase of CFU-C
formation in patients with congenital neutropenia. Furthermore, CFU-C
growth in both patients was supported when bone marrow cells were
preincubated with IL-3 in liquid culture followed by the stimulation with
G-CSF in semisolid agar culture. In contrast, that was not supported by the
preincubation with G-CSF and the subsequent stimulation with IL-3. This
evidence suggests that the hematopoietic progenitor cells in patients with
congenital neutropenia have the potential for developing CFU-C in the
combined stimulation with IL-3 and G-CSF, and that this growth may be
dependent on the priming of IL-3 followed by the stimulation with G-CSF.
The level of mature neutrophils in peripheral blood was not fully restored
to normal levels by the daily administration of G-CSF in doses of 100 to
200 micrograms/m2 of body surface area for 20 to 25 days in both patients.
These observations raise the possibility that the combination of IL-3 and
G-CSF might have a potential role for the increase of neutrophil counts in
patients with congenital neutropenia.
Volume 75,
Issue 11,
pp. 2143-2149,
06/01/1990
Copyright © 1990 by The American Society of Hematology
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
H. Kawaguchi, M. Kobayashi, K. Nakamura, N. Konishi, S.-i. Miyagawa, T. Sato, H. Toyoda, Y. Komada, S. Kojima, Y. Todoroki, et al.
Dysregulation of transcriptions in primary granule constituents during myeloid proliferation and differentiation in patients with severe congenital neutropenia
J. Leukoc. Biol.,
February 1, 2003;
73(2):
225 - 234.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. S. Grenda, S. E. Johnson, J. R. Mayer, M. L. McLemore, K. F. Benson, M. Horwitz, and D. C. Link
Mice expressing a neutrophil elastase mutation derived from patients with severe congenital neutropenia have normal granulopoiesis
Blood,
October 16, 2002;
100(9):
3221 - 3228.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. Nakamura, M. Kobayashi, N. Konishi, H. Kawaguchi, S.-i. Miyagawa, T. Sato, H. Toyoda, Y. Komada, S. Kojima, O. Katoh, et al.
Abnormalities of primitive myeloid progenitor cells expressing granulocyte colony-stimulating factor receptor in patients with severe congenital neutropenia
Blood,
December 15, 2000;
96(13):
4366 - 4369.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
N. Sawai, K. Koike, H. H. Mwamtemi, S. Ito, Y. Kurokawa, K. Sakashita, T. Kinoshita, T. Higuchi, K. Takeuchi, M. Shiohara, et al.
Thrombopoietin enhances neutrophil production by bone marrow hematopoietic progenitors with the aid of stem cell factor in congenital neutropenia
J. Leukoc. Biol.,
July 1, 2000;
68(1):
137 - 143.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
N. Konishi, M. Kobayashi, S.-i. Miyagawa, T. Sato, O. Katoh, and K. Ueda
Defective Proliferation of Primitive Myeloid Progenitor Cells in Patients With Severe Congenital Neutropenia
Blood,
December 15, 1999;
94(12):
4077 - 4083.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. C. Ward, Y. M. van Aesch, J. Gits, A. M. Schelen, J. P. de Koning, D. van Leeuwen, M. H. Freedman, and I. P. Touw
Novel Point Mutation in the Extracellular Domain of the Granulocyte Colony-stimulating Factor (G-CSF) Receptor in a Case of Severe Congenital Neutropenia Hyporesponsive to G-CSF Treatment
J. Exp. Med.,
August 16, 1999;
190(4):
497 - 508.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. G. Hunter and B. R. Avalos
Deletion of a Critical Internalization Domain in the G-CSFR in Acute Myelogenous Leukemia Preceded by Severe Congenital Neutropenia
Blood,
January 15, 1999;
93(2):
440 - 446.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
F. Dong, R. K. Brynes, N. Tidow, K. Welte, B. Lowenberg, and I. P. Touw
Mutations in the Gene for the Granulocyte Colony-Stimulating-Factor Receptor in Patients with Acute Myeloid Leukemia Preceded by Severe Congenital Neutropenia
N. Engl. J. Med.,
August 24, 1995;
333(8):
487 - 493.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. D. D'Andrea
Cytokine Receptors in Congenital Hematopoietic Disease
N. Engl. J. Med.,
March 24, 1994;
330(12):
839 - 846.
[Full Text]
|
|
|
|
|
