« on: August 05, 2008, 02:58:38 AM »
Treatment of -Thalassemia Patients with Recombinant Human Erythropoietin: Effect on Transfusion Requirements and Soluble Adhesion MoleculesAristeidis Chaidos, Alexandros Makis, Eleftheria Hatzimichael, Stavroula Tsiara, Maria Gouva, Evangelia Tzouvara, Konstantinos L. Bourantas
Department of Hematology, University of Ioannina Medical School, Ioannina, Greece
Key Words
Adhesion molecules
Endothelin
Erythropoietin
Recombinant human erythropoietin
Transfusion
-Thalassemia
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Abstract
The most common single genetic disorder and a major public health issue in Greece and other Mediterranean countries is -thalassemia. Current therapeutic approaches for homozygous -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 -thalassemia intermedia. Ten adult patients, 5 with -thalassemia major and 5 with -thalassemia intermedia, received 150 IU/kg rHuEPO (epoetin-) subcutaneously three times a week. Seven patients were transfused every 14-30 days and 3 with -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 -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.
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