Increase of F cells during acute hemolysis in glucose-6-phosphate dehydrogenase-deficient males.
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 +/- 0.24%) and F cell number (29 +/- 4.79%) 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 gamma chains. Globin chain electrophoresis showed that both G gamma and A gamma 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.
Schiliro'
G
G
Russo
A
A
Romeo
M A
MA
Testa
R
R
Musumeci
S
S
Mancuso
G R
GR
Russo
G
G
D000208:Acute Disease; D000743:Anemia, Hemolytic / Q000097:blood / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D004906:Erythrocyte Count; D004912:Erythrocytes / Q000473:pathology; D005260:Female; D005319:Fetal Hemoglobin / Q000032:analysis*; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000097:blood* / Q000150:complications; D006801:Humans; D007223:Infant; D008297:Male
D005319:Fetal Hemoglobin