Erythropoietin dose requirement in a patient with β-thalassaemia and chronic renal failure

Mm, Prata; Ja, Teixeira; Ft, de Sousa; Martins, Carolina Steller Wagner

doi:10.1093/ndt/9.8.1211b

Cited by 3 publications

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“…Winearls et al [21] used very high doses of EPO (600 IU/kg BW/week) to correct the anemia in an uremic patient with ·-thalassemia. Prata et al [5] treated a ß-thalassemia minor patient on dialysis with the initial EPO dose of 150 IU/kg BW/week, but progressively increased to 400 IU/kg BW/week without significant response; to avoid the need for transfusion, they used 800 IU/kg BW/week that allowed to obtain an Hb of 9.0-9.5. Such treatment did not have side effects and the arterial pressure was in the normal range.…”

Section: Resultsmentioning

confidence: 99%

“…In patients on hemodialysis affected by ß-thalassemia minor the EPO-related enhancement of effective erythropoiesis is dose dependent within the range of 15-500 IU/ kg BW/week [5,6,15,[20][21][22]. Although 95% of patients respond to the higher doses of EPO, the individual response is highly variable.…”

Section: Resultsmentioning

confidence: 99%

“…In addition, the individual response to EPO may be highly different [4]. It is well established that ß-thalassemia heterozygosis contributes to EPO resistance in dialysis [5,6]. Indeed, in patients with ß-thalassemia minor on chronic dialysis, higher EPO doses are needed to correct anemia even if all the known factors that contribute to EPO resistance are corrected [7].…”

Section: Introductionmentioning

confidence: 99%

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Relationship between Resistance to Erythropoietin and High Anomalous Hemoglobin Levels in Hemodialysis Patients with Beta-Thalassemia Minor

2003

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In dialysis patients β-thalassemia is a cause of resistance to erythropoietin (EPO). The aim of the present study is to evaluate the relationship between the amount of circulating anomalous hemoglobin chain and EPO resistance in hemodialysis. Ten hemodialyzed patients with β-thalassemia minor were studied. The mean hemoglobin level was 9.22 ± 0.91 g/dl, the HbA₂ ranging between 5.6 and 6.8%; the weekly EPO dose was 13,500 ± 7,185 IU/week and significantly correlated with HbA₂ (r = 0.965; p = 0.0001). When stratifying patients in two groups according to HbA₂ level (LOW <6%, n = 4; HIGH >6%, n = 6; HbA₂ levels, respectively, 5.7 ± 0.1 and 6.4 ± 0.3 g/dl, p = 0.002), it was evidenced that the need of EPO was 13,200 ± 3,033 IU/week in LOW and 36,167 ± 13,060 IU/week in HIGH (p < 0.001). The EPO Resistance Index in the two groups was 13.4 ± 4.1 IU/kg BW/week/g Hb in LOW and 21.9 ± 10.0 in HIGH (p < 0.05). No differences were evidenced between the two groups regarding age, dialysis, body weight, serum levels of urea nitrogen, creatinine, albumin, C-reactive protein, aluminum, ferritin, transferrin and parathyroid hormone. In conclusion, in patients with β-thalassemia minor on chronic hemodialysis, the amount of anomalous hemoglobin chain directly correlate with EPO dose, strongly indicating the magnitude of resistance to erythropoietin.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Relationship between Resistance to Erythropoietin and High Anomalous Hemoglobin Levels in Hemodialysis Patients with Beta-Thalassemia Minor

2003

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“…Due to this defi cient approach, the obtained Hb levels were constantly below 10 g/dl, although EPO was administered at very high doses, between 800 and 1,800 U/kg body weight/week i.v. [3,8] .…”

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confidence: 99%

Efficacy of Erythropoietin on Dialysis in Patients with Beta Thalassemia Minor

Iorio¹,

Nicola²,

Bellizzi³

et al. 2004

Blood Purif

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Background: It is unknown whether chronic erythropoietin (EPO) treatment is able to normalize hemoglobin (Hb) levels and ameliorate cardiac remodeling avoiding blood transfusions in uremic blood transfusion-dependent patients with β-thalassemia minor (β-thal). Methods: In 12 hemodialysis (HD) patients with β-thal, requiring blood transfusions despite EPO therapy, we planned to increase Hb levels up to the target levels (11–12 g/dl) within a one-year period by administering progressively higher doses of EPO (correction phase). We also planned to maintain the Hb target for an additional year (maintenance phase). Results: In the year before the study, patients required 3.3 ± 0.9 units of packed red blood cells. At baseline, the Hb level obtained with an EPO dose of 212 ± 73 U/kg/week i.v. was 8.2 ± 0.8 g/dl. The EPO dose was gradually increased within the first year up to 458 ± 78 U/kg/week at month 12 (correction phase) and then significantly tapered down during the maintenance phase (390 ± 54 U/kg/week at month 24). During the correction phase, the Hb levels markedly increased (11.1 ± 0.3 g/dl at month 12) and did not change in the maintenance phase. No blood transfusion was required throughout the 2 years of follow-up. Left ventricular (LV) mass index progressively decreased from the basal value of 144 ± 12 to 124 ± 11 g/m² in the first year and normalized in all patients at month 24 (109 ± 12 g/m², p < 0.001); this occurred in the absence of any change of LV cavity volume index (<90 ml/m²). Conclusions: In HD transfusion-dependent patients with β-thal, the administration of high EPO dose for 2 years permits the attainment and the maintenance of Hb targets without blood transfusions. This therapeutic approach permits a complete remission of concentric LV hypertrophy without any adverse effects on the vascular system.

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“…Although chronic renal failure, diabetes mellitus, and cardiac disorders have been reported in patients with thalassemia major, an association of chronic renal failure, diabetes mellitus, cardiomyopathy, and thalassemia major, as in our case, has not been reported in the literature. 1,[5][6][7][8][9][10][11][12]…”

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confidence: 99%

Silent Stroke in a Case of β-Thalassemia Major Associated With Chronic Renal Failure and Diabetes Mellitus

et al. 2003

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Severe anemia, growth retardation, diabetes mellitus, cardiac disorders, and, infrequently, stroke are well-known complications of thalassemia major. We report a girl, age 7 years, 2 months, with beta-thalassemia major associated with chronic renal failure, diabetes mellitus, and cardiomyopathy in whom a silent stroke was noted during follow-up. She was diagnosed with thalassemia major at age 6 months, chronic renal failure at age 3 years, 3 months, and diabetes mellitus and cardiomyopathy at age 7 years. Although cranial computed tomography was found to be normal at the age of 3 years, 3 months, magnetic resonance imaging showed cerebral infarct in the right frontal region at 7 years, 2 months. A thrombophilic panel revealed increased factor VIII and decreased protein C concentrations. She died from disseminated intravascular coagulation at age 7 years, 9 months. We did not record any clinical findings of stroke during her follow-up. We think that diabetes mellitus, dilated cardiomyopathy, and increased factor VIII and decreased protein C concentrations led to the occurrence of cerebral infarct. In conclusion, we emphasize that children with thalassemia major should be monitored closely for stroke. We also suggest that stroke can show a silent progression in severely affected children, as in our case.

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Erythropoietin dose requirement in a patient with β-thalassaemia and chronic renal failure

Cited by 3 publications

References 0 publications

Relationship between Resistance to Erythropoietin and High Anomalous Hemoglobin Levels in Hemodialysis Patients with Beta-Thalassemia Minor

Relationship between Resistance to Erythropoietin and High Anomalous Hemoglobin Levels in Hemodialysis Patients with Beta-Thalassemia Minor

Efficacy of Erythropoietin on Dialysis in Patients with Beta Thalassemia Minor

Silent Stroke in a Case of β-Thalassemia Major Associated With Chronic Renal Failure and Diabetes Mellitus

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