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  • Title: Correlation of in vitro enhancement of erythropoiesis and clinical response to steroids in Diamond-Blackfan anaemia.
    Author: Kalmanti M, Kalmantis T, Dimitriou H, Kattamis C.
    Journal: Haematologia (Budap); 1993; 25(4):263-9. PubMed ID: 8157208.
    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.
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