Ling Galina, Pinsk Vered, Golan-Tripto Inbal, Ling Eduard
Pediatric Gastroenterology and Nutrition Unit.
Pediatrics Department B, Saban Pediatric Medical Center for Israel, Soroka University Medical Center , Beer Sheva, Israel.
Hematol Rep. 2015 Sep 23;7(3):5987. doi: 10.4081/hr.2015.5987.
Congenital dyserythropoietic anemias (CDA) represent a heterogeneous group of disorders characterized by morphological abnormalities of erythroid precursor cells and various degrees of hemolysis. Iron overload is a result of continuous hemolysis and recurrent transfusions. It is treated with iron chelators, including deferasirox. We present here a case of acute liver failure in a 12 years old girl with CDA type I treated with deferasirox and discuss the approach to treatment.
先天性红细胞生成异常性贫血(CDA)是一组异质性疾病,其特征为红系前体细胞的形态异常和不同程度的溶血。铁过载是持续溶血和反复输血的结果。使用包括地拉罗司在内的铁螯合剂进行治疗。我们在此报告一例12岁I型CDA女童在接受地拉罗司治疗后发生急性肝衰竭的病例,并讨论其治疗方法。