Augmentation by Erythropoietin of the Fetal-Hemoglobin Response to Hydroxyurea in Sickle Cell Disease

• Published in: The New England journal of medicine Vol. 328; no. 2; pp. 73 - 80
• Main Authors: Rodgers, Griffin P, Dover, George J, Uyesaka, Nobuhiro, Noguchi, Constance T, Schechter, Alan N, Nienhuis, Arthur W
• Format: Journal Article
• Language: English
• Published: Boston, MA Massachusetts Medical Society 14.01.1993
• Subjects: Adult; Anemia; Anemia, Sickle Cell - blood; Anemia, Sickle Cell - drug therapy; beta-Thalassemia - blood; beta-Thalassemia - drug therapy; Bilirubin; Biological and medical sciences; Blood. Blood coagulation. Reticuloendothelial system; Bone marrow; Diabetes; Dietary supplements; Drug dosages; Drug Synergism; Drug Therapy, Combination; Erythrocyte Count; Erythrocyte Indices; Erythrocytes; Erythropoietin; Erythropoietin - therapeutic use; Ferrous Compounds - therapeutic use; Fetal Hemoglobin - biosynthesis; Fetuses; Hemoglobin; Hemolysis; Humans; Hydroxyurea; Hydroxyurea - therapeutic use; Intravenous administration; Iron; Kidney diseases; Laboratories; Laboratory animals; Male; Medical sciences; Patients; Pharmacology. Drug treatments; Polymerization; Recombinant Proteins - therapeutic use; Reticulocytes; Sickle cell anemia; Sickle cell disease; Sulfates; Thalassemia; Human; Hemoglobinopathy; Chemotherapy; Sickle cell anemia; Treatment; Erythropoietin; Exploration; Hemopathy; Fetal hemoglobin
• ISSN: 0028-4793; 1533-4406
• DOI: 10.1056/NEJM199301143280201

Stimulating fetal hemoglobin by increasing γ-globin synthesis in patients with sickle cell disease would, if the production of βS-globin decreased concomitantly, have a large “sparing” effect on the formation of intracellular hemoglobin S polymer 1 – 3 and would be expected to improve the acute and chronic hemolytic and vaso-occlusive complications of the disease. Azacytidine and hydroxyurea have been shown to increase fetal-hemoglobin levels in some patients with sickle cell disease 4 – 9 . When hydroxyurea is used to induce the production of fetal hemoglobin in sickle cell anemia, the responses of individual patients are variable, and prolonged treatment is required for . . .