Hematologic responses of patients with sickle cell disease to treatment with hydroxyurea

Article Abstract:

Fetal hemoglobin is different in structure from adult hemoglobin, the pigmented protein in the red blood cells that contains the iron and carries oxygen to body tissues. Because of this structural difference, fetal hemoglobin is not vulnerable to the genetic abnormality responsible for sickle cell disease, in which the red blood corpuscles take on a characteristic crescent shape. It has been found that increasing the level of fetal hemoglobin in the blood can partially counteract the disease process of sickle cell anemia. Hydroxyurea is a substance that stimulates production of fetal hemoglobin in persons with sickle cell disease. Ten hospitalized patients with sickle cell disease were treated with hydroxyurea for three months. Three of the ten showed no improvement, but the others experienced a 2- to 10-fold increase in fetal hemoglobin; the proportion of fetal to total hemoglobin rose from 1.6 percent to 6.8 percent on average. In four subjects, hydroxyurea treatment was discontinued for one to four months and then resumed; the increases in fetal hemoglobin were even greater than those realized after the initial treatment. In conclusion, hydroxyurea effectively stimulated production of fetal hemoglobin in a small number of patients with sickle cell disease. The clinical value of this treatment in sickle cell disease will not be known until large, controlled research studies are performed. (Consumer Summary produced by Reliance Medical Information, Inc.)

Abnormalities, Hemoglobin, Hemoglobins

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Normal fetal hemoglobin levels in the sudden infant death syndrome

Article Abstract:

Sudden infant death syndrome or SIDS is a leading cause of death in infants between 1 and 12 months of age. The diagnosis is made when all other possible reasons for death have been eliminated; this is called a diagnosis of exclusion. Various anatomic and biochemical differences between children who die of this condition and other children have been found. One possibility is that the level of fetal hemoglobin, the blood pigment responsible for the majority of oxygen transport in the body, is elevated in SIDS children. This study uses three different laboratory methods to measure the levels of fetal hemoglobin in SIDS infants and compares these levels to those of living infants, and infants who died of causes other than SIDS. There was no statistically significant difference in the quantity of fetal hemoglobin among these three groups of infants. Another test compared the percentage of fetal hemoglobin-containing red blood cells in mothers and fathers of SIDS infants and adult male and female controls. Again there were no significant differences among these groups. Fetal hemoglobin is neither accurate for measuring risk of SIDS in a living infant, nor is it usable as a marker of SIDS death.

Causes of, Sudden infant death syndrome

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Augmentation by erythropoietin of the fetal-hemoglobin response to hydroxyurea in sickle cell disease

Article Abstract:

Alternating hydroxyurea treatment with intravenous doses of recombinant erythropoietin in patients with sickle cell disease may be more effective and less toxic than treatment with hydroxyurea alone. Hydroxyurea increases fetal hemoglobin production, and recombinant erythropoietin has been found to increase the number of young red blood cells (reticulocytes) containing fetal hemoglobin. Increasing fetal hemoglobin is thought to improve blood circulation in patients with sickle cell disease. Four patients previously taking only hydroxyurea began alternating doses of erythropoietin and iron for three consecutive days with doses of hydroxyurea for four consecutive days. After seven weeks, the number of reticulocytes containing fetal hemoglobin rose 28%, and fetal hemoglobin levels rose an average of 48%. Side effects associated with erythropoietin were mild, and lower doses of potentially toxic hydroxyurea were needed to achieve results.

Erythropoietin, Recombinant, Recombinant erythropoietin

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