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副蛋白血症性纯红细胞再生障碍性贫血:1例患者的免疫学研究

Paraproteinemic variety of pure red cell aplasia: immunological studies in 1 patient.

作者信息

Resegotti L, Dolci C, Palestro G, Peschle C

出版信息

Acta Haematol. 1978;60(4):227-32. doi: 10.1159/000207718.

Abstract

A case of adult pure red cell aplasia (PRCA) with a serum IgG inhibitor to erythropoiesis and an IgG lambda M component is presented. The study of lymphocyte populations revealed a slight but definite decrease of E and EA rosettes, with dissociation between E rosettes and PHA blastic transformation of blood lymphocytes and increase of membrane IgM-bearing lymphocytes. The relationship between PRCA and paraproteinemia is discussed: it is suggested that the serum M component may derive from an immunological unbalance between T and B lymphocytes. Since a survey of the literature reveals 5 similar cases, it is suggested that paraproteinemia may be the hallmark of a particular variety of chronic PRCA 'type 1'.

摘要

本文报告一例成人纯红细胞再生障碍性贫血(PRCA),其血清中存在抑制红细胞生成的IgG抑制剂及IgG λ M成分。淋巴细胞群体研究显示E花环和EA花环略有但明确减少,E花环与血淋巴细胞PHA母细胞转化之间解离,且带有膜IgM的淋巴细胞增加。讨论了PRCA与副蛋白血症之间的关系:提示血清M成分可能源于T淋巴细胞和B淋巴细胞之间的免疫失衡。鉴于文献调查发现5例类似病例,提示副蛋白血症可能是一种特殊类型慢性PRCA“1型”的标志。

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