Document Detail


Long-term hydroxyurea therapy in beta-thalassaemia patients.
MedLine Citation:
PMID:  12605659     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: The study aimed to investigate the use of hydroxyurea (HU) for the treatment of beta-thalassaemia (beta-thal) patients. METHODS: We examined the haematological effects of orally administered HU (10-20 mg/kg/d) in 11 patients, including four beta-thal major and seven beta-thal intermedia patients. Complete blood count and levels of foetal haemoglobin (HbF), liver enzymes and serum creatinine were evaluated before and during HU. Response to therapy was evaluated at 6 months of treatment. RESULTS: A substantial increase in haemoglobin (Hb) level (4.1 g/dL), leading to complete withdrawal from a regular transfusion programme, was observed in one unique beta-thal major patient. In the beta-thal intermedia patients, increases in Hb level of 1.3, 1.9 and 2.0 g/dL were observed in three of seven (42.9%) patients during HU therapy. The mean values of Hb, mean corpuscular haemoglobin (MCH), and HbF were higher during HU treatment than baseline values (8.7 vs. 7.7 g/dL, P = 0.05; 26.7 vs. 22.9 pg, P = 0.05; 57.2 vs. 44.9%, P = 0.04; respectively). In contrast, the mean reticulocyte count measured during therapy decreased (97.0 x 10(9) vs. 632.0 x 10(9)/L, P = 0.03). No correlations were observed between levels of Hb and HbF (r = 0.77, P = 0.10), and levels of Hb and reticulocyte counts (r = 0.26, P = 0.31). No significant toxicity was observed in our patients. CONCLUSION: These results suggest that HU may improve Hb levels in beta-thal. Thus, we may conclude that a large trial concerning the response to HU in these patients should be carried out to clarify this issue.
Authors:
Erich Vinicius de Paula; Carmen Silvia Passos Lima; Valder Roberval Arruda; Fernando Lopes Alberto; Sara Teresinha Ollala Saad; Fernando Ferreira Costa
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Publication Detail:
Type:  Clinical Trial; Journal Article    
Journal Detail:
Title:  European journal of haematology     Volume:  70     ISSN:  0902-4441     ISO Abbreviation:  Eur. J. Haematol.     Publication Date:  2003 Mar 
Date Detail:
Created Date:  2003-02-27     Completed Date:  2003-05-09     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  8703985     Medline TA:  Eur J Haematol     Country:  Denmark    
Other Details:
Languages:  eng     Pagination:  151-5     Citation Subset:  IM    
Affiliation:
Haematology and Haemotherapy Centre, and Department of Internal Medicine, State University of Campinas, Campinas, São Paulo, Brazil.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Blood Cell Count
Clinical Enzyme Tests
Creatine / blood
Erythrocyte Indices / drug effects
Fetal Hemoglobin / drug effects
Hemoglobins / drug effects
Humans
Hydroxyurea / administration & dosage,  therapeutic use*,  toxicity
Liver / enzymology
Male
Reticulocyte Count
beta-Thalassemia / drug therapy*
Chemical
Reg. No./Substance:
0/Hemoglobins; 127-07-1/Hydroxyurea; 57-00-1/Creatine; 9034-63-3/Fetal Hemoglobin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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