Correlation of in vitro enhancement of erythropoiesis and clinical response to steroids in Diamond-Blackfan anaemia

Haematologia (Budap). 1993;25(4):263-9.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Cell Division / drug effects
  • Cells, Cultured
  • Child
  • Erythroid Precursor Cells / drug effects*
  • Erythropoiesis / physiology*
  • Fanconi Anemia / drug therapy*
  • Follow-Up Studies
  • Humans
  • Infant
  • Prognosis
  • Steroids / therapeutic use*

Substances

  • Steroids