Spontaneous erythroid colony formation in Brazilian patients with sickle cell disease

✍ Scribed by Perlingeiro, R.C.R.; Costa, F.F.; Saad, S.T.O.; Arruda, V.R.; Queiroz, M.L.S.

• Publisher: John Wiley and Sons
• Year: 1999
• Tongue: English
• Weight: 119 KB
• Volume: 61
• Category: Article
• ISSN: 0361-8609
• DOI: 10.1002/(sici)1096-8652(199905)61:1<40::aid-ajh8>3.0.co;2-0

✦ Synopsis

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 beta 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 10(5) 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/beta 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.