title: Blood mononuclear cells from patients with severe congenital neutropenia are capable of producing granulocyte colony-stimulating factor.
abstract: Severe congenital neutropenia (SCN) is a disorder of myelopoiesis characterized by severe neutropenia or absence of blood neutrophils secondary to a maturational arrest at the level of promyelocytes. We examined peripheral blood mononuclear cells (PBMC) of SCN patients who demonstrated normalization of their blood neutrophil counts in a phase II clinical study with recombinant human granulocyte colony-stimulating factor (rhG-CSF). When stimulated in vitro with bacterial lipopolysaccharides (LPS), PBMC of those SCN patients produced G-CSF activity, as judged by proliferation induction of the murine leukemia cell line, NFS-60. Western and Northern blot analysis showed G-CSF protein and G-CSF-mRNA indistinguishable in size from those of normal controls. We conclude that PBMC of the SCN patients tested are capable of synthesizing and secreting biologically active G-CSF in vitro.
authors.affiliation: Department of Pediatric Hematology and Oncology, Children's Hospital, Hannover Medical School, Germany.
authors.forename: T
authors.initials: T
authors.lastname: Pietsch
authors.forename: C
authors.initials: C
authors.lastname: Bührer
authors.forename: K
authors.initials: K
authors.lastname: Mempel
authors.forename: T
authors.initials: T
authors.lastname: Menzel
authors.forename: U
authors.initials: U
authors.lastname: Steffens
authors.forename: C
authors.initials: C
authors.lastname: Schrader
authors.forename: F
authors.initials: F
authors.lastname: Santos
authors.forename: C
authors.initials: C
authors.lastname: Zeidler
authors.forename: K
authors.initials: K
authors.lastname: Welte
mesh_terms: D000328:Adult; D015152:Blotting, Northern; D015153:Blotting, Western; D002452:Cell Count / Q000187:drug effects; D002648:Child; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000235:genetics / Q000378:metabolism* / Q000494:pharmacology; D006801:Humans; D007223:Infant; D007963:Leukocytes, Mononuclear / Q000187:drug effects / Q000378:metabolism* / Q000473:pathology; D008070:Lipopolysaccharides / Q000494:pharmacology; D008297:Male; D009503:Neutropenia / Q000097:blood / Q000151:congenital* / Q000378:metabolism / Q000473:pathology; D009504:Neutrophils / Q000187:drug effects; D012333:RNA, Messenger / Q000235:genetics / Q000378:metabolism; D011994:Recombinant Proteins / Q000494:pharmacology
chemical_list: D008070:Lipopolysaccharides; D012333:RNA, Messenger; D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor