Estudo comparativo do crescimento e diferenciação de progenitores eritroides do sangue periferico em pacientes eritrofalcemicos SS, SC e S/B falassemia

AUTOR(ES)
DATA DE PUBLICAÇÃO

1997

RESUMO

The ability of circulating progenitor cells from 69 patients with sickle cell disease (SCD) (42 SS, 14 SC and 13 S/? thalassemia) to develop erythroid colonies was studied in vitro in the presence or absence of growth factors (5637-CM and Epo). In both conditions, SCD patients presented significantly higher numbers of circulating burstforming unit-erythroid (BFU-E/5 x 105 MNC), when compared to control subjects (p <0.0001). We did not observe a significant difference in these responses among the genotypes SS, SC and S/? thalassemia. Moreover, there was a negative correlation between autocrine BFU-E and both hemoglobin and hematocrit levels (rs = - 0.3; p = 0.0065 and rs = - 0.27; P = 0.012, respectively). The study of the expression of growth factors receptors revealed an increased response to IL-3 (p <0.05), G-CSF (p <0.001) and Epo (p <0.02) in SCD patients. The expression of receptors for GM-CSF did not differ from that of the controls. We also observed a correlation between both stimulated (rs = 0.47; p = 0.014) and autocrine (rs = 0.46; p = 0.015) BFU-E and the expression of Epo receptors. On the other hand, the expression of G-CSF receptors showed a negative correlation with these BFU-E responses (rs = - 0.42; p = 0.04 and rs = - 0.57; p = 0.007, respectively). There was no correlation between HbF levels and any of the parameters evaluated. We observed no in vitro effects of the serum obtained from SCD patients on the growth and differentiation of hematopoietic precursors from normal human bone marrow. These results are of particular interest since they indicate that the phenomenon of spontaneous BFU-E derived colonies observed in SCD patients may be due to an increased expression of Epo and IL-3 receptors

ASSUNTO(S)

homoglobinopatia celulas - diferenciação eritropoese receptores de substancias endogenas celulas - proliferação anemia falciforme

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