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先天性红细胞生成异常性贫血：脾切除反应及无效红细胞生成的定量分析

Congenital dyserythropoietic anaemia: response to splenectomy and quantitation of ineffective erythropoiesis.

作者信息

Samson D, Halliday D, Chanarin I

出版信息

J Clin Pathol. 1977 Feb;30(2):184-90. doi: 10.1136/jcp.30.2.184.

DOI:10.1136/jcp.30.2.184
PMID:845266
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC476349/
Abstract

The clinical and haematological features of an unusual case of congenital dyserythropoietic anaemia are described. There was a pronounced haemolytic component to the anaemia, with a mean cell life of five days, and a remarkable response to splenectomy. Measurement of the incorporation of 15N glycine into the haem of circulating red cells and into bilirubin showed that haem turnover due to ineffective erythropoiesis was increased 45 times compared with a control group (11.63 mg/kg/day, NR = 0.26 + 0-10) and represented 51% of total erythroid haem turnover.

摘要

本文描述了一例罕见的先天性红细胞生成异常性贫血的临床和血液学特征。该贫血存在明显的溶血成分，平均红细胞寿命为5天，脾切除术后反应显著。测量15N甘氨酸掺入循环红细胞血红蛋白和胆红素中的情况，结果显示，与对照组相比，无效红细胞生成导致的血红蛋白更新增加了45倍（11.63mg/kg/天，正常范围=0.26±0.10），占红细胞系总血红蛋白更新的51%。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccde/476349/6c4c6b9a2a6f/jclinpath00160-0090-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccde/476349/65b3592df5fd/jclinpath00160-0087-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccde/476349/8bb6aa68dd23/jclinpath00160-0088-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccde/476349/6c4c6b9a2a6f/jclinpath00160-0090-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccde/476349/65b3592df5fd/jclinpath00160-0087-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccde/476349/8bb6aa68dd23/jclinpath00160-0088-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccde/476349/6c4c6b9a2a6f/jclinpath00160-0090-a.jpg

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Postgrad Med J. 1991 Apr;67(786):396-8. doi: 10.1136/pgmj.67.786.396.

本文引用的文献

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Electron and light microscopic study of the erythroblasts of patients with congenital dyserythropoietic anemia.先天性红细胞生成异常性贫血患者成红细胞的电子显微镜和光学显微镜研究
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