Spontaneous erythroid colony formation in Brazilian patients with sickle cell disease
R.C.R. Perlingeiro
Department of Physiology, State University of Campinas—UNICAMP, Campinas, Brazil
Search for more papers by this authorF.F. Costa
Department of Clinical Medicine, State University of Campinas—UNICAMP, Campinas, Brazil
Search for more papers by this authorS.T.O. Saad
Department of Pharmacology/Hemocentre, State University of Campinas, Campinas, Brazil
Search for more papers by this authorV.R. Arruda
Department of Clinical Medicine, State University of Campinas—UNICAMP, Campinas, Brazil
Search for more papers by this authorCorresponding Author
M.L.S. Queiroz
Department of Pharmacology/Hemocentre, State University of Campinas, Campinas, Brazil
Department of Pharmacology, Faculty of Medical Sciences, State University of Campinas—UNICAMP, P.O. Box 6111, CEP 13084-970, Campinas-SP, BrazilSearch for more papers by this authorR.C.R. Perlingeiro
Department of Physiology, State University of Campinas—UNICAMP, Campinas, Brazil
Search for more papers by this authorF.F. Costa
Department of Clinical Medicine, State University of Campinas—UNICAMP, Campinas, Brazil
Search for more papers by this authorS.T.O. Saad
Department of Pharmacology/Hemocentre, State University of Campinas, Campinas, Brazil
Search for more papers by this authorV.R. Arruda
Department of Clinical Medicine, State University of Campinas—UNICAMP, Campinas, Brazil
Search for more papers by this authorCorresponding Author
M.L.S. Queiroz
Department of Pharmacology/Hemocentre, State University of Campinas, Campinas, Brazil
Department of Pharmacology, Faculty of Medical Sciences, State University of Campinas—UNICAMP, P.O. Box 6111, CEP 13084-970, Campinas-SP, BrazilSearch for more papers by this authorAbstract
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 × 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. Am. J. Hematol. 61:40–45, 1999. © 1999 Wiley-Liss, Inc.
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