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Blood, 15 December 2000, Vol. 96, No. 13, pp. 4366-4369
BRIEF REPORT
Abnormalities of primitive myeloid progenitor cells expressing
granulocyte colony-stimulating factor receptor in patients with severe
congenital neutropenia
Kazuhiro Nakamura,
Masao Kobayashi,
Nakao Konishi,
Hiroshi Kawaguchi,
Shin-ichiro Miyagawa,
Takashi Sato,
Hidemi Toyoda,
Yoshihiro Komada,
Seiji Kojima,
Osamu Katoh, and
Kazuhiro Ueda
From the Department of Pediatrics, School of Medicine,
the Department of Child Health, Faculty of Education, and the
Department of Environment and Mutation, Research Institute for
Radiation Biology and Medicine, Hiroshima University, Hiroshima, Japan;
the Department of Pediatrics, Mie University School of Medicine, Tsu,
Japan; and the Department of Developmental Pediatrics, Nagoya
University School of Medicine, Nagoya, Japan.
To define the basis for faulty granulopoiesis in patients with
severe congenital neutropenia (SCN), the expression of granulocyte colony-stimulating factor receptor (G-CSFR) in primitive myeloid progenitor cells and their responsiveness to hematopoietic factors were
studied. Flow cytometric analysis of bone marrow cells based on the
expression of CD34, Kit receptor, and G-CSFR demonstrated a reduced
frequency of CD34+/Kit+/ G-CSFR+
cells in patients with SCN. The granulocyte-macrophage colony formation
of CD34+/Kit+/G-CSFR+ cells in
patients was markedly decreased in response to G-CSF alone and to the
combination of stem cell factor, the ligand for flk2/flt3, and IL-3
with or without G-CSF in serum-deprived semisolid culture. In contrast,
no difference in the responsiveness of
CD34+/Kit+/G-CSFR cells was noted
between patients with SCN and subjects without SCN. These results
demonstrate that the presence of qualitative and quantitative
abnormalities of primitive myeloid progenitor cells expressing G-CSFR
may play an important role in the impairment of granulopoiesis in
patients with SCN.
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