title: Impaired production of GM-CSA in bone marrow and peripheral blood monocytes in two patients with severe congenital neutropenia.
abstract: The capacity of bone marrow and peripheral blood cells to stimulate colony formation by normal granulocyte-macrophage progenitor cells (CFU-GM) was investigated in two patients with severe congenital neutropenia using soft agar-gel culture techniques. In both patients, monocyte/macrophage-derived colony stimulating activity (GM-CSA) in conditioned medium was found to be significantly decreased compared to the control. Furthermore, the capacity of patients' unfractionated peripheral blood leukocytes to stimulate normal CFU-GM in overlayers, was also found significantly decreased. In contrast, the number of bone marrow and peripheral blood CFU-GM was within the normal range in both patients. Patients' CFU-GM showed a normal pattern of in vitro differentiation when they were stimulated by exogenous GM-CSA. These data indicate that in some cases of severe congenital neutropenia, monocyte/macrophage-derived GM-CSA may be impaired. Whether this abnormality plays some role in the regulation of granulopoiesis in these patients is unclear.
authors.affiliation: Division of Hematology, University of Crete School of Medicine, University Hospital, Greece.
authors.forename: G
authors.initials: G
authors.lastname: Eliopoulos
authors.forename: S
authors.initials: S
authors.lastname: Coulocheri
authors.forename: A
authors.initials: A
authors.lastname: Eliopoulos
authors.forename: G
authors.initials: G
authors.lastname: Vaiopoulos
authors.forename: C
authors.initials: C
authors.lastname: Tsantali
authors.forename: N
authors.initials: N
authors.lastname: Anagnou
mesh_terms: D000380:Agranulocytosis / Q000097:blood / Q000209:etiology*; D001853:Bone Marrow / Q000378:metabolism*; D002469:Cell Separation; D002675:Child, Preschool; D005260:Female; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000096:biosynthesis*; D006801:Humans; D007223:Infant; D008297:Male; D009000:Monocytes / Q000378:metabolism*; D009503:Neutropenia / Q000151:congenital / Q000378:metabolism*
chemical_list: D016178:Granulocyte-Macrophage Colony-Stimulating Factor