Cohen A, Martin M, Schwartz E
Br J Haematol. 1984 Oct;58(2):369-73. doi: 10.1111/j.1365-2141.1984.tb06096.x.
To determine the therapeutic effect of long-term, intensive iron chelation therapy, we studied liver iron content and histology in four children with thalassaemia major during 52-83 months of intensive therapy with desferrioxamine. The initial biopsies obtained prior to or within 21 months after beginning chelation therapy had Grade IV iron staining, with heavy iron deposition present in parenchymal and reticuloendothelial cells. Subsequent biopsies, obtained when serum ferritin levels had fallen to 71-246 micrograms/l, contained Grade 0 or Grade I stainable iron. Little or no iron was present in parenchymal or reticuloendothelial cells. The liver iron concentration, measured by magnetic susceptibility, returned to normal or nearly normal levels. Hepatic fibrosis did not progress during treatment with desferrioxamine. These findings demonstrate that intensive and sustained chelation therapy with desferrioxamine will remove excessive liver iron and preserve hepatocellular structure.
为了确定长期强化铁螯合疗法的治疗效果,我们研究了4例重型地中海贫血患儿在接受去铁胺强化治疗52 - 83个月期间的肝脏铁含量和组织学情况。在开始螯合治疗前或治疗后21个月内进行的初始活检显示为IV级铁染色,实质细胞和网状内皮细胞中有大量铁沉积。随后在血清铁蛋白水平降至71 - 246微克/升时进行的活检显示可染色铁为0级或I级。实质细胞或网状内皮细胞中几乎没有铁。通过磁化率测量的肝脏铁浓度恢复到正常或接近正常水平。在用去铁胺治疗期间,肝纤维化没有进展。这些发现表明,去铁胺强化持续螯合疗法将去除肝脏中过多的铁并保留肝细胞结构。