The usage of human recombinant erythropoietin (rhEPO) in βthalassemia shows in many sudies, that not only it increases the synthesis of γ chains, but it also improves erythroid parameters. Among our population of thalassemic patients, eight patients with βthalassemia intermedia that were regulary transfused, were given escalating doses of rhEPO mostly darpoietin-α. Before receiving rhEPO, all patients had undergone splenectomy, also received iron chelation therapy, and folic acid supplementation. No patient received iron supplementation together with rhEPO.

With rhEPO, two patients were able to discontinue their requirements for transfusions. In the others, haemoglobin level was maintained above 8 g/dl. Four patients experienced bone pain which was successfully treated with hydroxyurea. All patients had improvement in their quality of life. This small series illustrates that rhEPO may be useful and cost effective in the long term (considering the risks of red blood cell transfusion) in some selected splenectomized transfusion-dependent patients with βthalassemia intermedia.

Results

Transfusion requirements (ml/kg/year)
PatientsYear of birthSexFinal doses of rhEPO (UI*kg/week)Follow-up (months)BeforeAfterHydroxyurea
* interruption of rhEPO because of participation to ICL 670 A 0107 protocol 
1 1975 M 970 33 150 75 + 
2 1973 F 1365 34 125 0 + 
3 1965 M 820 33 130 0 + 
4 1959 F 650 27 125 98 + 
5 1979 F 715 15 210 160 * + 
6 1978 M 1035 7 255 90 − 
7 1987 F 450 3 135 80 − 
8 1974 M 900 7 190 75 + 
Transfusion requirements (ml/kg/year)
PatientsYear of birthSexFinal doses of rhEPO (UI*kg/week)Follow-up (months)BeforeAfterHydroxyurea
* interruption of rhEPO because of participation to ICL 670 A 0107 protocol 
1 1975 M 970 33 150 75 + 
2 1973 F 1365 34 125 0 + 
3 1965 M 820 33 130 0 + 
4 1959 F 650 27 125 98 + 
5 1979 F 715 15 210 160 * + 
6 1978 M 1035 7 255 90 − 
7 1987 F 450 3 135 80 − 
8 1974 M 900 7 190 75 + 
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Topics:
transfusion, hydroxyurea, blood transfusion, bone pain, erythrocyte transfusion, follow-up, hemoglobin measurement, iron, iron chelation therapy, recombinant erythropoietin

Author notes

Corresponding author

2005, The American Society of Hematology
2004
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