A high level of fetal hemoglobin was found in an 8-yr-old boy without any hematologic disorders except for a moderate anemia. The absence of hemoglobin abnormalities in the parents led us to suspect a latent malignant disease that, on follow-up, was confirmed to be myelomonocytic leukemia. Hemoglobin biosynthetic studies provided evidence of unbalanced synthesis of globin subunits by reticulocy...

Induction of fetal hemoglobin (HbF) has therapeutic importance for patients with beta-hemoglobin disorders. Previous studies showed that let-7 microRNAs (miRNAs) are highly regulated in erythroid cells during the fetal-to-adult developmental transition, and that targeting let-7 mediated the up-regulation of HbF to greater than 30% of the total globin levels in human adult cultured erythroblasts...

By PHAEDON FESSAS AND GEORGE STAMATOYANNOPOULOS H EREDITARY PERSISTENCE of fetal hemoglobin was described in a Greek family in 1961 and included the combination of this anomaly with aand with -thalassemia.1’2 Since then, further cases or families have been observed in Greece, two of which have been reported in whole or in part in Greek medical literature.3’4 This material now permits an adequat...

The fetal-to-adult hemoglobin switch and silencing of fetal hemoglobin (HbF) have been areas of long-standing interest among hematologists, given the fact that clinical induction of HbF production holds tremendous promise to ameliorate the clinical symptoms of sickle cell disease (SCD) and β-thalassemia. In this article, we discuss historic attempts to induce HbF that have resulted in some ther...

Background: β-thalassemia is one of the most widespread disease in the world, including Iran. In this study, we reported, for the first time, A 290-bp β-globin gene deletion in the south of Iran. Methods: Four individuals from three unrelated families with Arabic ethnic background were studied in Khuzestan Province. Red blood cell indices and hemoglobin analysis were carried out accor...

INTRODUCTION Iron is an essential micronutrient in the growing fetus. OBJECTIVE The purpose of this study is to find the possible correlations that may exist between maternal and fetal iron status and newborn weight. MATERIAL AND METHODS The study included 97 mothers scheduled to give birth by elective caesarean section in the central maternity of Tébessa (east of Algeria) between January a...

Many human aneuploidy syndromes have unique phenotypic consequences, but in most instances it is unclear whether these phenotypes are attributable to alterations in the dosage of specific genes. In human trisomy 13, there is delayed switching and persistence of fetal hemoglobin (HbF) and elevation of embryonic hemoglobin in newborns. Using partial trisomy cases, we mapped this trait to chromoso...

Hydroxyurea is the first drug that, under well-organized, large-scale trials in adults, has shown a beneficial effect on the clinical course of sickle cell disease. Several small-scale trials have been conducted in children, but they used different therapeutic schedules, and only one was a single-blind crossover trial. Still, children are clearly good responders to the treatment because a rapid...

Studies of fetal hemoglobin and bone marrow ultrastructure were performed on a patient with a recently described syndrome consisting of lymphosarcoma, short stature, defective cellular Immunity, hypogammaglobulinemia, and mosaicism for a marker chromosome. Abnormalities of the erythroid cells, including multinucleated erythroblasts, nuclear bodies, megaloblasts, and frayed cytoplasm, were seen ...

To study the effect of genetic differences in the maternal oxyhemoglobin dissociation curve on fetal O2 supply, we compared eight pregnant ewes homozygous for high O2 affinity hemoglobin (A) with eight pregnant ewes homozygous for low O2 affinity hemoglobin (B). Each ewe carried a single fetus. Fetal weights were not significantly different (A, 3,000 +/- 170 g; B, 3,070 +/- 270 g). The A ewes h...