title: Correlation of in vitro enhancement of erythropoiesis and clinical response to steroids in Diamond-Blackfan anaemia.
abstract: To investigate whether the clinical response to steroids in Diamond-Blackfan anaemia (DBA) can be predicted in vitro, erythroid cultures from six patients was performed. The increment of burst forming unit erythroid (BFU-E) and colony forming unit erythroid (CFU-E) derived colonies had been studied with the in vitro addition of steroids. Our results showed: 1) a relative insensitivity to low and optimum concentration of erythropoietin (Epo) in cultures, and 2) lower CFU-E and BFU-E colony formation in all cases studied. (CFU-Es of normal controls/10(5) cells; were 85.6, BFU-Es 37.8, while the patients' CFU-E were 18.1 and BFU-E 7/10(5) cells with two IU of Epo) with poor or no growth in three cases, 3) increment up to 60-110% with in vitro addition of steroids in three cases, 4) high serum Epo levels in all Diamond-Blackfan anaemia patients, and 5) clinical response to steroids in the three patients who responded in vitro.
authors.affiliation: Dept. of Pediatric Hematology-Oncology, University Hospital, University of Crete Medical School, Iraklion.
authors.forename: M
authors.initials: M
authors.lastname: Kalmanti
authors.forename: T
authors.initials: T
authors.lastname: Kalmantis
authors.forename: H
authors.initials: H
authors.lastname: Dimitriou
authors.forename: C
authors.initials: C
authors.lastname: Kattamis
mesh_terms: D000328:Adult; D002455:Cell Division / Q000187:drug effects; D002478:Cells, Cultured; D002648:Child; D015672:Erythroid Precursor Cells / Q000187:drug effects*; D004920:Erythropoiesis / Q000502:physiology*; D005199:Fanconi Anemia / Q000188:drug therapy*; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D011379:Prognosis; D013256:Steroids / Q000627:therapeutic use*
chemical_list: D013256:Steroids