Defects of Granulopoiesis in Patients with Severe Congenital Neutropenia
スポンサーリンク
概要
- 論文の詳細を見る
To confirm the abnormalities of primitive myeloid progenitor cells in patients withsevere congenital neutropenia (SCN), we studied their responsiveness to hematopoieticfactors including granulocyte colony-stimulating factor (G-CSF). In all SCN patientsstudied no abnormalities of granulocyte colony-stimulating factor receptor (G-CSFR) genewere detected by polymerase chain reaction-single-strand conformation polymorphism analysisand sequence analysis. A flow cytometric analysis of bone marrow cells based on theexpression of CD34, Kit receptor, and G-CSFR demonstrated a reduced frequency ofCD34+/Kit+/G-CSFR+ cells in patients with SCN. The granulocyte/macrophage (GM)-colonyformation of CD34+/Kit+/G-CSFR+ cells in patients was markedly decreased at allconcentrations of G-CSF in serum-deprived semisolid culture. The responsiveness ofCD34+/Kit+/G-CSFR+ cells in patients showed a reduced response to the combination of stemcell factor, the ligand for flk2/flt3, and interleukin-3 with or without G-CSF inserum-deprived semisolid and liquid suspension cultures. In contrast, no difference in theresponsiveness of CD34+/Kit+/G-CSFR- cells was noted between SCN patients and normalsubjects. The bone marrow cells from a patient who underwent bone marrow transplantationshowed a restoration of both the reduced frequency and the decreased level of GM-colonyformation of CD34+/Kit+/G-CSFR+ cells. These results demonstrate that the presence ofqualitative and quantitative abnormalities of primitive myeloid progenitor cells expressingG-CSFR may play an important role in the impairment of granulopoiesis in patients with SCN.
- 広島大学の論文
- 2002-09-10
著者
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Kazuhiro Nakamura
1) Department Of Pediatrics Hiroshima University School Of Medicine Hiroshima734-8551 Japan 2) Depar
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Nakamura Kazuhiro
1) Department of Pediatrics, Hiroshima University School of Medicine, Hiroshima734-8551, Japan 2) Department of Child Health, Hiroshima University Graduate School ofEducation, Hiroshima, Japan 3) Department of Cellular and Molecular Biology, ResearchInsti