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• W1567102430 abstract "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." @default.
• W1567102430 created "2016-06-24" @default.
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• W1567102430 date "2000-12-15" @default.
• W1567102430 modified "2023-09-27" @default.
• W1567102430 title "Abnormalities of primitive myeloid progenitor cells expressing granulocyte colony-stimulating factor receptor in patients with severe congenital neutropenia" @default.
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• W1567102430 doi "https://doi.org/10.1182/blood.v96.13.4366.h8004366_4366_4369" @default.
• W1567102430 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/11110716" @default.
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