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Abnormalities of primitive myeloid progenitor cells expressing granulocyte colony-stimulating factor receptor in patients with severe congenital neutropenia

NAKAMURA, Kazuhiro ; KOBAYASHI, Masao ; UEDA, Kazuhiro ; KONISHI, Nakao ; KAWAGUCHI, Hiroshi ; MIYAGAWA, Shin-Ichiro ; SATO, Takashi ; TOYODA, Hidemi ; KOMADA, Yoshihiro ; KOJIMA, Seiji ; KATOH, Osamu

Blood, 2000-12, Vol.96 (13), p.4366-4369 [Peer Reviewed Journal]

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Title:
Abnormalities of primitive myeloid progenitor cells expressing granulocyte colony-stimulating factor receptor in patients with severe congenital neutropenia
Author: NAKAMURA, Kazuhiro ; KOBAYASHI, Masao ; UEDA, Kazuhiro ; KONISHI, Nakao ; KAWAGUCHI, Hiroshi ; MIYAGAWA, Shin-Ichiro ; SATO, Takashi ; TOYODA, Hidemi ; KOMADA, Yoshihiro ; KOJIMA, Seiji ; KATOH, Osamu
Subjects: Antigens, CD34 - analysis ; Biological and medical sciences ; Bone Marrow - pathology ; Cell Differentiation ; Clone Cells ; Culture Media, Serum-Free ; Drug Synergism ; Flow Cytometry ; Genetic Predisposition to Disease ; Granulocyte Colony-Stimulating Factor - pharmacology ; Hematopoietic Stem Cells - drug effects ; Hematopoietic Stem Cells - metabolism ; Hematopoietic Stem Cells - pathology ; Humans ; Immunodeficiencies ; Immunodeficiencies. Immunoglobulinopathies ; Immunopathology ; Infections - etiology ; Interleukin-3 - pharmacology ; Medical sciences ; Membrane Proteins - pharmacology ; Myeloid Cells - drug effects ; Myeloid Cells - metabolism ; Myeloid Cells - pathology ; Neutropenia - congenital ; Neutropenia - genetics ; Neutropenia - pathology ; Proto-Oncogene Proteins c-kit - analysis ; Receptors, Granulocyte Colony-Stimulating Factor - physiology ; Recombinant Proteins - pharmacology ; Recurrence ; Stem Cell Factor - pharmacology
Is Part Of: Blood, 2000-12, Vol.96 (13), p.4366-4369
Description: 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. (Blood. 2000;96:4366-4369)
Publisher: Washington, DC: The Americain Society of Hematology
Language: English
Identifier: ISSN: 0006-4971
EISSN: 1528-0020
DOI: 10.1182/blood.v96.13.4366
PMID: 11110716
Source: Alma/SFX Local Collection

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Abnormalities of primitive myeloid progenitor cells expressing granulocyte colony-stimulating factor receptor in patients with severe congenital neutropenia

NAKAMURA, Kazuhiro ; KOBAYASHI, Masao ; UEDA, Kazuhiro ; KONISHI, Nakao ; KAWAGUCHI, Hiroshi ; MIYAGAWA, Shin-Ichiro ; SATO, Takashi ; TOYODA, Hidemi ; KOMADA, Yoshihiro ; KOJIMA, Seiji ; KATOH, Osamu

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