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[血管免疫母细胞性腺病]

[Angioimmunoblastic adenopathies].

作者信息

Mathé G, Amiel J L, Gérard-Marchant R, Caillou B, Pico J L, Machover D

出版信息

Nouv Presse Med. 1976 Jun 12;5(24):1515-8.

PMID:940740
Abstract

Angio-lymphoblastic lymphadenopathies are a newly described haematological entity, though not rare, characterised by a histological triad (vascular neogenesis, highly polymorphic or predominantly immunoblastic cellular proliferation and the presence of acidophilic protein deposits), a clinical syndrome consisting essentially of voluminous disseminated lymphadenopathy and hepatosplenomegaly and abnormal laboratory findings dominated by a polyclonal dysproteinaemia. This disorder, the first four French cases of which are described here, does not appear to be malignant in its early stages and is sensitive to corticosteroid therapy at that time. Secondary sarcomatous transformation is possible.

摘要

血管淋巴母细胞性淋巴结病是一种新描述的血液学实体,虽不罕见,其特征为组织学三联征(血管生成、高度多形性或主要为免疫母细胞性细胞增殖以及嗜酸性蛋白沉积的存在)、一种主要由大量播散性淋巴结病和肝脾肿大组成的临床综合征以及以多克隆性蛋白异常血症为主的异常实验室检查结果。本文描述了法国最初的4例该疾病,其早期阶段似乎并非恶性,且此时对皮质类固醇治疗敏感。继发性肉瘤样转化是可能的。

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