The pharmacological manipulation of fetal haemoglobin: trials using hydroxyurea and recombinant human erythropoietin

Acta Haematologica
M A El-HazmiF al-Mohareb

Abstract

Hydroxyurea (HU) and recombinant human erythropoietin (rHuEpo) have been used in several studies to elevate Hb F level in sickle cell disease (SCD) patients and hence to ameliorate the clinical presentations of the disease. The treatment protocol and doses have varied in the different studies. We studied the effects of HU+rHuEpo combination therapy in sickle cell anaemia (SCA patients) to investigate the Hb F manipulation and hence treatment of SCA. Six patients with severe SCA were selected for treatment with HU (20-25 mg/kg body weight) and rHuEpo (400-800 U/kg body weight) combination therapy for 4 weeks followed by HU (20-25 mg/kg body weight) maintenance therapy for 6 months to 1 year. Iron and folic acid were administered during HU+rHuEpo treatment. Signs, symptoms and complications were recorded to obtain the severity index. Only patients with a severity index > or = 6 were included in the study. Haematological and biochemical parameter values, Hb A2, Hb F, Hb F distribution, Hb F cells, bilirubin level and reticulocyte count were assessed at least on 2-3 occasions prior to initiation of the therapy protocol to establish baseline values. During the treatment period, the clinical presentations were monitored and the estim...Continue Reading

Citations

Jan 25, 2003·British Journal of Haematology·Christina Halsey, Irene A Roberts
Sep 12, 2001·Medicine·M H Steinberg, G P Rodgers
Oct 3, 2002·Annual Review of Medicine·Martin H Steinberg, Carlo Brugnara
Dec 27, 2011·The Indian Journal of Medical Research·Mohsen A F El-HazmiArjumand S Warsy
May 25, 2007·Thrombosis Research·J F A NoronhaH Z W Grotto
Feb 19, 2009·British Journal of Haematology·Jane Hankins, Banu Aygun
Jan 30, 2003·American Journal of Hematology·Martin H SteinbergFranca Barton
Sep 24, 1999·Pediatric Pulmonology·J KnightI Browning

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