Five male Sicilian children with glucose-6-phosphate dehydrogenase deficiency were studied shortly after hemolytic crisis in order to evaluate the immediate effects of massive hemolysis on fetal Hb (HbF) levels and the number of circulating F cells. Hematological values seen 4 months after the children recovered from the crisis were considered representative of the patients' steady state. All patients had an increase in HbF levels (2.26 Â± 0.24Â±) and F cell number (29 Â± 4.79Â±) in the acute phase and their HbF values and F cells returned to normal range at control. Globin synthesis was balanced in the peripheral blood and bone marrow and there was a small peak of Î³ chains. Globin chain electrophoresis showed that both G Î³ and A Î³ genes were active in all patients. These results confirm that hemolytic stress produces increased F cell release in peripheral blood. Such release is rapid enough (less than 72 h) to be consistent with the hypothesis of an induction of HbF synthesis in late erythroid precursors. Â© 1985 International Pediatric Research Foundation, Inc.
|Titolo:||Increase of F cells during acute hemolysis in glucose-6-phosphate dehydrogenase-deficient males|
|Data di pubblicazione:||1985|
|Appare nelle tipologie:||1.1 Articolo in rivista|