II型先天性红细胞生成异常性贫血中无效红细胞生成和外周溶血的研究。

Studies of ineffective erythropoiesis and peripheral haemolysis in congenital dyserythropoietic anaemia type II.

作者信息

Barosi G, Cazzola M, Stefanelli M, Ascari E

出版信息

Br J Haematol. 1979 Oct;43(2):243-50. doi: 10.1111/j.1365-2141.1979.tb03747.x.

DOI:10.1111/j.1365-2141.1979.tb03747.x

Abstract

Erythrokinetic parameters were estimated in six patients suffering from congenital dyserythropoietic anaemia type II (CDA II) by means of a mathematical model of iron kinetics. A wide variation in effectiveness of erythroid activity was observed, and a significant negative correlation was found between ineffective erythropoiesis and peripheral haemolysis. In four patients with prominent peripheral haemolysis, splenectomy was carried out. Marked improvement in their clinical condition and in haemoglobin level resulted.

摘要

通过铁动力学数学模型，对6例II型先天性红细胞生成异常性贫血（CDA II）患者的红细胞生成动力学参数进行了评估。观察到红系活性的有效性存在很大差异，且无效红细胞生成与外周溶血之间存在显著负相关。对4例外周溶血明显的患者实施了脾切除术。结果其临床状况和血红蛋白水平均有显著改善。