Diamond-Blackfan syndrome: a possible role of cellular factors for erythropoietic suppression.
CFU-E growth from fractionated bone marrow cells of 5 children with Diamond-Blackfan syndrome was studied. In all patients, CFU-E growth was reduced in mononuclear cell-rich fraction. In 2 of the 5 patients, CFU-E growth was returned to normal by the depletion of E-rosette forming cells or monocytes from mononuclear cell-rich fraction. In the patient whose CFU-E growth returned to normal by the depletion of E-rosette forming cells, co-cultivation between bone marrow buffy coat cells and autologous bone marrow E-rosette forming cells resulted in a significant decrease of CFU-E growth, and there was a significant increase in CFU-E growth by treatment with monoclonal antiserum to OKT4. We concluded that immunologic causes such as cellular factors may play a role, at least in part, in the pathogenesis of Diamond-Blackfan syndrome.
D000741:Anemia, Aplastic / Q000097:blood / Q000473:pathology*; D000911:Antibodies, Monoclonal / Q000276:immunology; D001854:Bone Marrow Cells; D002450:Cell Communication; D002469:Cell Separation; D002478:Cells, Cultured; D002675:Child, Preschool; D003114:Colony-Forming Units Assay; D003720:Densitometry; D004920:Erythropoiesis; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008214:Lymphocytes / Q000276:immunology; D008297:Male; D009000:Monocytes / Q000276:immunology; D012010:Red-Cell Aplasia, Pure / Q000097:blood / Q000473:pathology*; D012397:Rosette Formation
D000911:Antibodies, Monoclonal