Hydroxyurea in treatment of sickle-cell anaemia

N. S. Smetanina, E. V. Rumyantseva, A. Kutlar, Yu N. Tokarev

Research output: Contribution to journalArticlepeer-review

Abstract

The study was aimed at determining the relative amounts of the total γ-,Gγ-andAγ-globin messenger ribonucleic acids (mRNA), foetal haemoglobin (Hb F), and theGγ-andAγ-globin chains in its composition in 52 patients suffering from sickle-cell anaemia with various haplotypes. The Hb F level was shown to depend upon the haplotype. The amount of γ-globin mRNA corresponded to the Hb F level, with the amount of mRNA considerably exceeding the Hb F level in patients more than ten years old, which might apparently be related to a decrease in translation with age. The relative amount ofGγ-globin mRNAs corresponded to the number of theGγ-globin chains. Of the examined patients, twenty-two subjects received hydroxyurea at a daily dose of 10–12 mg/kg for from 6 to 24 months. On the background of carried out therapy, the patients showed a statistically reliable increase in the levels of Hb (from 79 to 88 g/l; p < 0.01), Hb F (from 4.8 to 14.3%; p < 0.0005), MCV (from 93.6 to 109.4 fl p < 0.0005), and MCH (from 322 to 368 g/l; p < 0.0005), to be accompanied by a decrease in the number of reticulocytes (from 278.5 × 109/l to 189.6 × 109/l; p < 0.01), leukocytes (from 13.4 × 109/l to 10.9 × 109/l; p < 0.05). The degree of the Hb F increase was observed to depend on the βS-haplotype. Six patients had accompanying α+-thalassemia (-α3.7/αα). No differences in the Hb F increment level on the background of the carried out therapy between the patients with and without α-thalassemia were revealed. The dynamics of the relative amount of γ-globin mRNA showed that long-term administration of permanent-dose hydroxyurea in patients with sickle-cell anaemia leads to an increase in the transcription activity of γ-globin genes, a decrease in the amount of the «silent» γ-globin mRNA, and to an alteration in the ratio of theGγ/Aγ-globin chains in the composition of HbF.

Original languageEnglish (US)
Pages (from-to)22-26
Number of pages5
JournalPediatric Hematology/Oncology and Immunopathology
Volume2
Issue number1
StatePublished - 2003

Keywords

  • Foetal haemoglobin
  • Hydroxyurea
  • Sickle-cell anaemia
  • Thalassemia

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Immunology and Allergy
  • Immunology
  • Hematology
  • Oncology

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