Fetal hemoglobin and hemolysis markers in sickle cell anemia

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Fetal hemoglobin and hemolysis markers in sickle cell anemia☆

It has been known for many years that high levels of fetal hemoglobin (Hb F) have an important clinical benefit in patients with sickle cell anemia (SCA). This knowledge was initially based on the observation that populations of Indian and Arabian SCA patients with high Hb F levels have a milder clinical form of the disease.1 Later on, epidemiological studies demonstrated that patients with Hb ...

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Fetal hemoglobin in sickle cell anemia

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...

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Fetal hemoglobin in sickle cell anemia: relationship to erythrocyte adhesion markers and adhesion.

To assess whether fetal hemoglobin (HbF) modulates the adhesion of sickle erythrocytes to endothelium, children with homozygous sickle cell anemia (SS disease) were studied, using this physiologically crucial period to evaluate the relationships between HbF and the major erythrocyte adhesion markers. The mean level of CD36(+) erythrocytes was 2.59% +/- 2.15% (+/- SD, n = 40) with an inverse rel...

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Fetal hemoglobin in sickle cell anemia: a glass half full?

Fetal hemoglobin (HbF) modulates the phenotype of sickle cell anemia by inhibiting deoxy sickle hemoglobin (HbS) polymerization. The blood concentration of HbF, or the number of cells with detectable HbF (F-cells), does not measure the amount of HbF/F-cell. Even patients with high HbF can have severe disease because HbF is unevenly distributed among F-cells, and some cells might have insufficie...

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Hydroxyurea enhances fetal hemoglobin production in sickle cell anemia.

Hydroxyurea, a widely used cytotoxic/cytostatic agent that does not influence methylation of DNA bases, increases fetal hemoglobin production in anemic monkeys. To determine its effect in sickle cell anemia, we treated two patients with a total of four, 5-d courses (50 mg/kg per d, divided into three oral doses). With each course, fetal reticulocytes increased within 48-72 h, peaked in 7-11 d, ...

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ژورنال

عنوان ژورنال: Revista Brasileira de Hematologia e Hemoterapia

سال: 2015

ISSN: 1516-8484

DOI: 10.1016/j.bjhh.2015.03.015