Difference in pattern of erythropoietin response between β-thalassemia/hemoglobin e children and adults

Abstract

Thalassemia is one of the most common genetic disorders in Thailand. The thalassemic patients have many pathophysiologic changes secondary to chronic anemia. During these last few years there have been many trials to cure or improve the anemic condition in thalassemia by using various agents, including erythropoietin (EPO). Thus it is very important to understand the EPO response to different degree of anemia in the thalassemic patients. In this study we evaluated the EPO status in 53 β-thalassemia/HbE patients, from 4-61 years old, by enzyme-linked immunosorbent assay. The results showed that the levels of EPO in β-thalassemia/HbE patients were much higher than in normal control subjects: mean ± SE = 527 ± 183.20 and 3 45 ± 0.47 mIU/ml respectively. The reverse correlation between the levels of EPO and hematocrit (r = -0.704) was also observed. There was also a tendency to have higher levels of EPO in β-thal/HbE children than in adults, although this was statistically insignificant The observed versus predicted levels of EPO (log O/P ratio) showed that most patients had good EPO response to the degree of anemia. However, inappropriate decrease of EPO response was observed in 8/40 adult patients. The EPO levels in these patients were not correlated with any physical or laboratory studies, including kidney function. We thus piopose that if EPO is to be considered as one of the alternative treatment to the thalassemic patients, in the future, it may benefit only the patients with low EPO levels.