background: &beta-thalassemia; is the most common monogenic disorder in human. the (ct) polymorphism at -158 upstream region of the &gammag-globin; gene and pharmacological factors such as hydroxyurea have been reported to influence &gamma-globin; gene expression and the severity of clinical symptoms of &beta-thalassemia.; methods: in the present study, 51 &beta-thalassemia; intermediate patie...

Abstract Background Delta beta (δβ) thalassemia is an unusual variant of thalassemia with elevated level of fetal hemoglobin (HbF). Homozygous patients of this disorder, unlike β-thalassemia, show mild anemia. Only few cases of δβ-thalassemia have been reported from India in the available indexed English literature. Case presentation A four-year old male child was evaluated for recent-onset...

Homozygous sickle cell disease in the eastern province of Saudi Arabia is clinically mild. Circulating fetal hemoglobin levels of 16.0 +/- 7.4% were found in these anemic patients, but only 1.09 +/- 0.97% in their sickle trait parents. To determine whether these sickle cell anemia patients inherit an increased capacity to synthesize fetal hemoglobin, a radioimmunoassay of fetal and adult hemogl...

Baboons exposed to acute hemolytic stres increase their production of fetal hemoglobin (HbF). Although the maximal in vivo HbF levels attained in 5 treated animals varied from 6.4% to 34.8%, their cultured bone marrow erythroid cells reverted to the fetal pattern of hemoglobin synthesis. These data suggest that HbF synthesis is modulated by the interaction of inhibiting and promoting factors, w...

hemoglobin f (hbf, α 2 γ 2 ) is a major contributor to the clinical heterogeneity and ameliorating agent observed in patients with the β-globin disorders including β-thalassemia and sickle cell disease (scd). during fetal life, hbf is the major hemoglobin but is largely substituted by adult hemoglobin (hba, α 2 β 2 ) following a globin expression switch after birth. increased γ-globin expressio...

objective: the use of fetal hemoglobin (hbf) inducer drugs is considered as a novel approach in treatment of β-hemoglobinopathies, especially β- thalassemia and sickle cell disease. hbf inducers including hydroxyurea, histone deacetylase (hdac) inhibitor agents such as sodium butyrate, azacitidine, decitabine and new immunomodulator drugs like pomalidomide, lenalidomide and thalidomide can redu...

background: when fetal red cells enter the maternal circulation from placenta, an event would be happened that is described as feto-maternal hemorrhage (fmh). this life-threatening condition could be detected by using rbc antigens (surface antigens and intracellular antigens). therefore, the measurement of fetal rbc in an artificial model would be useful to calculate fmh and consequently the do...

Reactivation of fetal hemoglobin (HbF) expression is an important therapeutic option in patients with hemoglobin disorders. In sickle cell disease (SCD), an increase in HbF inhibits the polymerization of sickle hemoglobin and the resulting pathophysiology. Hydroxyurea, an inducer of HbF, has already been approved for the treatment of patients with moderate and/or severe SCD. Recent clinical tri...

BACKGROUND Delta beta (δβ) thalassemia is an unusual variant of thalassemia with elevated level of fetal hemoglobin (HbF). Homozygous patients of this disorder, unlike β-thalassemia, show mild anemia. Only few cases of δβ-thalassemia have been reported from India in the available indexed English literature. CASE PRESENTATION A four-year old male child was evaluated for recent-onset jaundice. ...

Fetal hemoglobin (HbF) is the major genetic modulator of the hematologic and clinical features of sickle cell disease, an effect mediated by its exclusion from the sickle hemoglobin polymer. Fetal hemoglobin genes are genetically regulated, and the level of HbF and its distribution among sickle erythrocytes is highly variable. Some patients with sickle cell disease have exceptionally high level...