Giordano P C, Huisman W, Harteveld C L
Hemoglobinopathies Laboratory, Department of Human and Clinical Genetics, Leiden University Medical Center, Einthovenweg 20, 2300RC Leiden, The Netherlands.
ISRN Hematol. 2011;2011:473152. doi: 10.5402/2011/473152. Epub 2011 Jul 5.
We report some observations from our laboratory practice that might be important for the treatment of sickle cell disease (SCD). We describe data from two cases indicating that iron depletion might have a beneficial effect diminishing the formation of HbS in favor of HbF, possibly reducing the severity of the disease. We believe that it would be worthwhile to monitor the course of the disease comparing cases with identical genotypes with and without iron depletion, and we advise to consider chelation therapy to reduce iron overload in patients with SCD.
我们报告了一些来自实验室实践的观察结果，这些结果可能对镰状细胞病（SCD）的治疗具有重要意义。我们描述了两个病例的数据，表明铁耗竭可能具有有益作用，可减少HbS的形成，有利于HbF的生成，可能减轻疾病的严重程度。我们认为，比较具有相同基因型且有无铁耗竭的病例来监测疾病进程是值得的，并且我们建议考虑采用螯合疗法来减少SCD患者的铁过载。
