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[淋巴系统综合征期间获得性C1酯酶抑制剂缺乏症]

[Acquired C1-esterase inhibitor deficiencies during lymphoid syndromes].

作者信息

Oberling F, Hauptmann G, Lang J M, Bergerat J P, Mayer G, Batzenschlager A, Hammann B, Gillet B

出版信息

Nouv Presse Med. 1975 Nov 8;4(38):2705-8.

PMID:812065
Abstract

Very marked abnormalities of the complement system were discovered in two patients suffering from a lymphoid syndrome and an IgM 7S dysglobulinaemia. The abnormalities in the complement system were related to a deficiency in C1-estérase (C1 INH). Several findings suggest that such a deficiency is acquired, in particular the absence of any family history of angio-neurotic oedema and, above all, the detection of a marked fall in levels of the C1 fraction which does not exist in the congenital form of deficiency of the inhibitor. The IgM 7S immunoglobulins found in the serum of both patients are probably responsible for the abnormalities in the complement system observed. Such acquired deficiencies in C1 INH are extremely rare since only a few cases have been reported in the literaute, in particular two cases in patients with lymphosarcoma with a serum IgM 7S.

摘要

在两名患有淋巴样综合征和IgM 7S球蛋白异常血症的患者中发现了补体系统的非常明显的异常。补体系统的异常与C1酯酶(C1 INH)缺乏有关。多项研究结果表明,这种缺乏是后天获得的,特别是不存在血管神经性水肿的家族病史,最重要的是,检测到C1组分水平显著下降,而先天性抑制剂缺乏症中不存在这种情况。在两名患者血清中发现的IgM 7S免疫球蛋白可能是观察到的补体系统异常的原因。这种后天获得的C1 INH缺乏极为罕见,因为文献中仅报道了少数病例,特别是两名患有血清IgM 7S的淋巴肉瘤患者。

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