Correlation of in vitro enhancement of erythropoiesis and clinical response to steroids in Diamond-Blackfan anaemia.
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.
Kalmanti
M
M
Dept. of Pediatric Hematology-Oncology, University Hospital, University of Crete Medical School, Iraklion.
Kalmantis
T
T
Dimitriou
H
H
Kattamis
C
C
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*
D013256:Steroids