Document Detail


Treatment of beta-thalassemia patients with recombinant human erythropoietin: effect on transfusion requirements and soluble adhesion molecules.
MedLine Citation:
PMID:  15153710     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The most common single genetic disorder and a major public health issue in Greece and other Mediterranean countries is beta-thalassemia. Current therapeutic approaches for homozygous beta-thalassemia entail blood transfusions and iron chelation therapy with deferoxamine or deferiprone for preventing tissue hemosiderosis. Recently, much effort has focused on various inducers of fetal hemoglobin (HbF) such as recombinant human erythropoietin (rHuEPO), especially in beta-thalassemia intermedia. Ten adult patients, 5 with beta-thalassemia major and 5 with beta-thalassemia intermedia, received 150 IU/kg rHuEPO (epoetin-alpha) subcutaneously three times a week. Seven patients were transfused every 14-30 days and 3 with beta-thalassemia intermedia were only occasionally transfused. The minimum duration of treatment was 12 weeks in order to define if there was any response. Transfusion intervals were modified according to the rHuEPO response to maintain stable Hb values. Lower transfusion requirements were observed in 5 patients after rHuEPO treatment (p = 0.028). In the 3 non-transfused patients, Hb values increased, and the patients are still being treated and followed up for a period ranging from 14 weeks to 2 years. Two patients with thalassemia major discontinued treatment after 12 weeks, as they did not achieve any response regarding transfusion requirements or Hb values. Pretreatment serum transferrin receptor levels were higher than in controls (p < 0.001) and significantly increased following rHuEPO treatment (p = 0.027). Patients had higher serum endothelin-3, sICAM-1 and sE-selectin values before rHuEPO treatment compared to controls (p < 0.001, p < 0.001 and p = 0.016, respectively), but these values were not altered during treatment. HbF values presented a slight, non-significant increase. rHuEPO treatment has a beneficial effect in transfusion-dependent beta-thalassemia patients. Although a slight increase in HbF levels was observed, other possible mechanisms are probably involved. None of our patients experienced thrombotic complications and a rise in blood pressure.
Authors:
Aristeidis Chaidos; Alexandros Makis; Eleftheria Hatzimichael; Stavroula Tsiara; Maria Gouva; Evangelia Tzouvara; Konstantinos L Bourantas
Publication Detail:
Type:  Clinical Trial; Controlled Clinical Trial; Journal Article    
Journal Detail:
Title:  Acta haematologica     Volume:  111     ISSN:  0001-5792     ISO Abbreviation:  Acta Haematol.     Publication Date:  2004  
Date Detail:
Created Date:  2004-05-21     Completed Date:  2004-06-15     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0141053     Medline TA:  Acta Haematol     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  189-95     Citation Subset:  IM    
Copyright Information:
Copyright 2004 S. Karger AG, Basel
Affiliation:
Department of Hematology, University of Ioannina Medical School, Ioannina, Greece.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Blood Transfusion / utilization*
Cell Adhesion Molecules / blood*,  drug effects
E-Selectin / blood,  drug effects
Endothelin-3 / blood,  drug effects
Erythropoietin, Recombinant / administration & dosage*
Female
Fetal Hemoglobin / analysis,  drug effects
Hemoglobins / analysis,  drug effects
Humans
Intercellular Adhesion Molecule-1 / blood,  drug effects
Male
Middle Aged
Receptors, Transferrin / blood,  drug effects
Solubility
Treatment Outcome
beta-Thalassemia / complications,  drug therapy*,  therapy
Chemical
Reg. No./Substance:
0/Cell Adhesion Molecules; 0/E-Selectin; 0/Endothelin-3; 0/Erythropoietin, Recombinant; 0/Hemoglobins; 0/Receptors, Transferrin; 126547-89-5/Intercellular Adhesion Molecule-1; 9034-63-3/Fetal Hemoglobin

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