Abstract

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 
1975 970 33 150 75 
1973 1365 34 125 
1965 820 33 130 
1959 650 27 125 98 
1979 715 15 210 160 * 
1978 1035 255 90 − 
1987 450 135 80 − 
1974 900 190 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 
1975 970 33 150 75 
1973 1365 34 125 
1965 820 33 130 
1959 650 27 125 98 
1979 715 15 210 160 * 
1978 1035 255 90 − 
1987 450 135 80 − 
1974 900 190 75 

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