Clinical and Hematological Response to Hydroxyurea in a Patient with Hb Leporbp-Thalassemia

The possibility of increasing Hb F in vivo using drugs like 5-azacy-tidine, hydroxyurea, and butyrate has been established. However, in many cases this does not entail an increase in total hemoglobin. We report on a patient with Hb Lepore/β-thalassemia being treated with hydroxyurea (30 mg/Kg/day) b...

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Bibliographic Details
Published in:Hemoglobin 1997, Vol.21 (3), p.219-226
Main Authors: Rigano, P., Manfré, L., Galla, R. La, Renda, D., Renda, M. C., Calabrese, A., Calzolari, R., Maggio, A.
Format: Article
Language:English
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Summary:The possibility of increasing Hb F in vivo using drugs like 5-azacy-tidine, hydroxyurea, and butyrate has been established. However, in many cases this does not entail an increase in total hemoglobin. We report on a patient with Hb Lepore/β-thalassemia being treated with hydroxyurea (30 mg/Kg/day) because of the presence of erythroid extra-medullary masses with severe neurological abnormalities. During therapy the patient showed a remarkable improvement in neurological signs due to the reduction in extra-medullary masses, a significant increase in both total hemoglobin (from 5.8 to 9.7 g/dl) and Hb F (from 4.9 g/dl to 9.1 g/dl). The marked improvement in hemoglobin level in our patient with Hb Lepore/β-thalassemia suggests γ-globin gene activation due to the DNA structure determined by the crossover event.
ISSN:0363-0269
1532-432X
DOI:10.3109/03630269708997382