Spontaneous erythroid colony formation in Brazilian patients with sickle cell disease

R. C R Perlingeiro, F. F. Costa, S. T O Saad, V. R. Arruda, Mary L S Queiroz

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

The ability of circulating progenitor cells to develop erythroid colonies was studied in vitro in the presence or absence of growth factors (5637-CM and erythropoietin) in 63 patients with sickle cell disease (SCD) (36 homozygotes for hemoglobin [Hb] S, 13 double heterozygotes for Hb S and β thalassemia, and 14 SC patients) in Southeast Brazil. In the presence of growth factors, SCD patients (all genotypes) presented significantly higher numbers of circulating burst-forming unit-erythroid (BFU-E/5 x 105 MNC), when compared with control subjects. However, when the progenitor cells were cultured in the absence of added stimulus, high numbers of BFU-E were observed only in the genotypes SS and S/β thalassemia. SC patients presented a similar response to the control subjects. Moreover, there was an inverse correlation between spontaneous (without stimulus) BFU-E and Hb levels in SCD patients. These results suggest that the formation of spontaneous BFU-E observed in SCD may be due to an expanded erythropoiesis secondary to hemolysis.

Original languageEnglish (US)
Pages (from-to)40-45
Number of pages6
JournalAmerican Journal of Hematology
Volume61
Issue number1
DOIs
StatePublished - 1999

Keywords

  • Autoproliferation
  • BFU-E
  • Hematopoiesis
  • Sickle cell disease

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