Unal Sule, Hazirolan Tuncay, Eldem Gonca, Gumruk Fatma
Division of Pediatric Hematology, Hacettepe University, Ankara, Turkey.
Pediatr Hematol Oncol. 2011 Apr;28(3):217-21. doi: 10.3109/08880018.2010.522230. Epub 2010 Nov 17.
The iron loading related to erythrocyte transfusions is the major cause of morbidities and mortalities in patients with β-thalassemia major (β-TM). Deferasirox, an orally active iron chelator, has been reported to cause serum creatinine increases in addition to acute renal failures in elderly patients with comorbidities. The nefrotoxicities in patients using deferasirox, despite the facts that the drug is minimally excreted from kidneys and its effective chelation of iron from liver and heart, may rise the question of decomparmentalization of iron from these organs to kidneys. Thirteen patients with β-TM were included in the study (mean age 18.5 ± 7.5 years [9-33 years]). The patients received deferasirox in a dose of 34.3 ± 6.5 mg/kg [17-37 mg/kg]. Four patients (31%) exhibited consecutive increases in serum creatinine greater than 33% above baseline twice during the follow-up period. The results indicated that the earliest iron chelation starts in liver in patients receiving deferasirox. Additionally, by the 6th month of deferasirox, the status of cardiac and renal iron in chronically transfused patients with β-TM were preserved. This may indicate that the serum creatinine increases may not be attributed to iron decompartmantalization from other organs to kidneys.
与红细胞输血相关的铁负荷是重型β地中海贫血（β-TM）患者发病和死亡的主要原因。地拉罗司是一种口服活性铁螯合剂，据报道，除了会导致患有合并症的老年患者出现急性肾衰竭外，还会引起血清肌酐升高。尽管地拉罗司从肾脏排泄极少且能有效螯合肝脏和心脏中的铁，但使用该药物的患者出现肾毒性可能会引发从这些器官到肾脏的铁去分隔化问题。本研究纳入了13例β-TM患者（平均年龄18.5±7.5岁[9 - 33岁]）。患者接受地拉罗司的剂量为34.3±6.5mg/kg[17 - 37mg/kg]。4例患者（31%）在随访期间血清肌酐连续两次升高超过基线水平33%以上。结果表明，接受地拉罗司治疗的患者最早在肝脏开始铁螯合。此外，在地拉罗司治疗6个月时，长期输血的β-TM患者的心脏和肾脏铁状态得以维持。这可能表明血清肌酐升高可能并非归因于铁从其他器官到肾脏的去分隔化。
