Pediatric Onco-Hematology, Stem Cell Transplantation and Cellular Therapy Division, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria Città della Salute e della Scienza, University of Torino, Torino, Italy.
Department of Pediatric and Public Health Sciences, University of Turin, Turin, Italy.
Br J Haematol. 2022 Oct;199(2):171-172. doi: 10.1111/bjh.18393. Epub 2022 Aug 5.
Transfusion-dependent Diamond-Blackfan anaemia (DBA) patients rapidly develop iron overload and frequently experience cardiac complications. The report by Lecornec and colleagues offers useful details on indications and the management of deferiprone, a highly efficient chelator in removing excess cardiac iron but associated with a high risk of agranulocytosis in DBA patients. Commentary on: Lecornec et al. Agranulocytosis in patients with Diamond-Blackfan anaemia (DBA) treated with deferiprone for post-transfusion iron overload: A retrospective study of the French DBA cohort. British Journal of Haematology 2022;199:285-288.
输血依赖型 Diamond-Blackfan 贫血（DBA）患者迅速发生铁过载，并经常出现心脏并发症。 Lecornec 等人的报告提供了有关去铁酮适应证和管理的有用细节，去铁酮是一种高效的螯合剂，可去除多余的心脏铁，但与 DBA 患者的粒细胞缺乏症风险较高相关。述评：Lecornec 等人。用去铁酮治疗输血后铁过载的 Diamond-Blackfan 贫血（DBA）患者的粒细胞缺乏症：法国 DBA 队列的回顾性研究。英国血液学杂志 2022；199：285-288。
