Hausmann K
Haematol Blood Transfus. 1978;21:145-61.
Knowledge of disturbancies of iron utilization has been considerably extended by histochemical-ultrastructural findings and the results of immunoradiometric assays for serum ferritin. -- In chronic anaemia due to infections or neoplastic diseases hyposideraemia and normal unsaturated iron binding capacity were associated with increased iron retention in macrophages and slightly to highly increased serum ferritin (500--4000 ng/ml). -- 117 patients with sideroblastic anaemia formed a heterogenous group of diverse aetiology. The iron granules of ringed sideroblasts contained nonferritin iron in mitochondria. At diagnosis, a normal iron status was found in single cases. More frequently, praelatent and latent iron overload with ferritin levels up to more than 2000 ng/ml were observed. Manifest iron overload with tissue damage was mostly the result of numerous transfusions (ferritin 4700 bis 9500 ng/ml). -- After i.v. application of colloidal iron endothelial siderosis was a regular finding. The typical uniform granules representing nonferritin-iron in lysosomes disappeared in the course of 1--3 years completely. In contrast, the colloidal iron taken up simultaneously by the macrophages was rapidly transformed into ferritin and easily used up for haemoglobin synthesis when required. The corresponding increase of serum ferritin up to maximal 4000 mg/ml was dose related. Continued blood losses lead to residual endothelial siderosis after exhaustion of macrophageal iron and recurrence of iron deficiency anaemia. The serum ferritin fell to low levels (0--12 ng/ml) as observed in untreated cases.
组织化学超微结构研究结果以及血清铁蛋白免疫放射分析结果极大地拓展了我们对铁利用障碍的认识。——在由感染或肿瘤疾病引起的慢性贫血中,低铁血症和正常的不饱和铁结合能力与巨噬细胞中铁潴留增加以及血清铁蛋白轻度至高度升高(500 - 4000 ng/ml)相关。——117例铁粒幼细胞贫血患者病因各异,构成了一个异质性群体。环形铁粒幼细胞的铁颗粒在线粒体中含有非铁蛋白铁。诊断时,个别病例铁状态正常。更常见的是,观察到潜在和潜伏性铁过载,铁蛋白水平高达2000 ng/ml以上。明显的铁过载伴组织损伤主要是多次输血的结果(铁蛋白4700至9500 ng/ml)。——静脉注射胶体铁后,内皮细胞铁沉积是常见现象。溶酶体中代表非铁蛋白铁的典型均匀颗粒在1 - 3年内完全消失。相比之下,巨噬细胞同时摄取的胶体铁迅速转化为铁蛋白,并在需要时很容易用于血红蛋白合成。血清铁蛋白相应升高至最高4000 mg/ml与剂量相关。持续失血导致巨噬细胞铁耗尽后残留内皮细胞铁沉积,并使缺铁性贫血复发。血清铁蛋白降至低水平(0 - 12 ng/ml),这与未治疗病例中观察到的情况相同。