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伴有肺部浸润、低补体血症和血管炎的免疫母细胞性淋巴结病。一种高免疫综合征。

Immunoblastic lymphadenopathy with pulmonary infiltrates, hypocomplementemia and vasculitis. A hyperimmune syndrome.

作者信息

Weisenburger D, Armitage J, Dick F

出版信息

Am J Med. 1977 Dec;63(6):849-54. doi: 10.1016/0002-9343(77)90535-6.

Abstract

A detailed description of the clinical and morphologic characteristics of four patients with immunoblastic lymphadenopathy, pulmonary infiltrates, hypocomplementemia and vasculitis is presented. Noteworthy in the patients described here is the clinical similarity to patients with collagen-vascular diseases and systemic drug reactions. The frequent occurrence of pulmonary symptoms, bilateral interstitial infiltrates and pleural effusions is emphasized. The morphology suggests stimulation of the immune system by antigenic agents, and the low complement levels and the presence of vasculitis suggest that circulating immune complexes may be present. Immunoblastic lymphadenopathy may represent a syndrome in which the adenopathy is but a nonspecific part of a systemic hyperimmune response to unknown antigens. The clinical course of the patients reviewed suggests that supportive treatment and corticosteroids constitute the safest therapeutic approach.

摘要

本文详细描述了4例具有免疫母细胞性淋巴结病、肺部浸润、补体血症低下及血管炎的患者的临床和形态学特征。此处所描述患者值得注意的是其与胶原血管病及全身性药物反应患者在临床上的相似性。文中强调了肺部症状、双侧间质性浸润及胸腔积液的频繁出现。形态学表现提示抗原性物质刺激免疫系统,而补体水平低下及血管炎的存在提示可能存在循环免疫复合物。免疫母细胞性淋巴结病可能代表一种综合征,其中淋巴结病只是对未知抗原的全身性超免疫反应的非特异性部分。所回顾患者的临床病程表明,支持性治疗和皮质类固醇构成最安全的治疗方法。

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