Spontaneous erythroid colony formation in Brazilian patients with sickle cell disease.

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 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.

journal_name

Am J Hematol

authors

Perlingeiro RC,Costa FF,Saad ST,Arruda VR,Queiroz ML

doi

10.1002/(sici)1096-8652(199905)61:1<40::aid-ajh8>3

keywords:

subject

Has Abstract

pub_date

1999-05-01 00:00:00

pages

40-5

issue

1

eissn

0361-8609

issn

1096-8652

pii

10.1002/(SICI)1096-8652(199905)61:1<40::AID-AJH8>3

journal_volume

61

pub_type

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