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[因C1酯酶抑制剂后天缺乏导致的后天性血管神经性水肿伴发淋巴增殖综合征。附病例报告及文献复习]

[Acquired angioneurotic edema caused by acquired deficiency of C1 esterase inhibitor disclosing lymphoproliferative syndrome. Apropos of a case, review of the literature].

作者信息

Chevrant-Breton J, Mazéas D, Bagot M, Henry M, Goasguen J, Robin J P

出版信息

Ann Dermatol Venereol. 1982;109(12):1049-56.

PMID:7171202
Abstract

One case of acquired angio-neurotic oedema is described and discussed with the other cases recorded in literature since Caldwell's one in 1972. This entity is characterized by: --the late onset of angio-oedema but its presence only in about half cases, --a complement deficiency resulting from the lack of C1-esterase inhibitor, --the absence of familial identical cases, --the great frequency of associated illnesses overall lympho-proliferative diseases, --the therapeutic response to either etiologic treatment of the associated disease or to the symptomatic effect of drugs used in hereditary angio-neurotic oedema.

摘要

本文描述并讨论了1例获得性血管神经性水肿病例,并与自1972年考德威尔报道以来文献中记录的其他病例进行了比较。该病症具有以下特征:——血管性水肿发病较晚,但仅约半数病例出现;——由于缺乏C1酯酶抑制剂导致补体缺乏;——无家族性相同病例;——总体上与淋巴增殖性疾病相关的疾病发生率很高;——对相关疾病的病因治疗或遗传性血管神经性水肿所用药物的对症治疗有治疗反应。

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