Abstract

Abstract BACKGROUND AND AIMS Thalassemia is a group of hemoglobinopathies characterized by the decrease or lack of β or α globin chain production (Hb) [1]. Minor thalassemia has little to no symptoms since the other gene is capable of compensating for the anomaly; however, it represents a rare cause of resistance to erythropoietin (EPO) in chronic hemodialysis patients (CHP) in multiple regions, particularly the Mediterranean region [2]. As a matter of fact, CHP suffering from minor thalassemia often take higher EPO doses to correct the anemia [3], even if all causes of EPO resistance are resolved; a recent Italian investigation indicated that EPO need in this population is almost double in comparison with other groups [4]. EPO résistance can be secondary to the quantitative anomalies of the hemoglobin chain, notably Hb A2 and Hb F, Biagio Di Lorio et al. reported a positive correlation between the percentage of Hb A2 and EPO doses [5], actually patients that had an Hb A2 >6% needed higher EPO doses and vice versa. The aims of this study were to evaluate the efficiency of different therapeutic means [Erythropoiesis-stimulating agents (ESA), blood transfusion (TS)] aiming to correct the anemia in CHP with minor thalassemia. To detect the correlation between quantitative anomalies in hemoglobin chains and EPO resistance. METHOD It's a descriptive multi-centric study, involves eight CHP suffering from minor thalassemia. The statistical analysis of the data was done through Excel using the correlation coefficient and SPSS using the P-value. A value <.05 was considered as significant. RESULTS The average value of hemoglobin was 7.35 g/dL, Ferritin (M: 964 ± 971 ng/L) and PTH levels (M: 433 ± 126 pg/mL). These results are detailed in Table 1. Electrophoresis was done for all our patients with an average of HbA à 96.2 ± 1.7%; HbA2 à 3.43 ± 1.41%; HbF à 0.35 ± 0.45%. We have noticed that a decreased percentage up to nul of Hb F fraction is correlated with the lowest Hb levels (Fig. 1). The main therapeutic mean in anemia patients is epoietin beta, the average dose given to patients is 300 UI/kg/week, 326UI/kg/s at 2 months, 233 UI/kg/s at 6 months and 326 UI/kg/s at 12 months. Figure 2 illustrates the response to the EPO during the maintenance phase. The Darbepoetin alpha (μg/Kg/s) is administered (depending on availability) to two patients out of the eight. One of the two was receiving a dose of 0.57 μg/kg/s for 8 weeks, the other one was receiving a dose 0.7 μg/kg/s for 8 weeks as well, with a different response in the two patients. CONCLUSION: The presence of minor thalassemia in chronic hemodialysis patients entails erythropoietin resistance and a serious case of anemia. In our study, the maximum EPO dose used was 500 UI/kg/s reaching a value of 9.7 g/dL of Hb.

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