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与 Good 综合征相关的肉芽肿性淋巴细胞性间质性肺病对免疫球蛋白治疗有反应。

Granulomatous-lymphocytic Interstitial Lung Disease Associated with Good's Syndrome That Responded to Immunoglobulin Therapy.

机构信息

Department of Respiratory Medicine, Saiseikai Yokohamashi Tobu Hospital, Japan.

Department of Diagnostic Pathology, Saiseikai Yokohamashi Tobu Hospital, Japan.

出版信息

Intern Med. 2021 Oct 1;60(19):3137-3142. doi: 10.2169/internalmedicine.6456-20. Epub 2021 Mar 15.

Abstract

Good's syndrome is associated with thymoma and acquired immunodeficiency. A 54-year-old man visited our hospital with a complaint of cough. Chest imaging revealed diffuse nodular shadows and anterior mediastinal mass. Hypogammaglobulinemia and a decreased B lymphocyte count were found by a laboratory evaluation. The lung nodules markedly regressed after immunoglobulin therapy. The mediastinal mass and remaining nodule were surgically resected and diagnosed as a type AB thymoma and a necrotizing epithelioid granuloma with T lymphocyte-dominant alveolitis, respectively. The overall appearances of these lesions were mostly in line with the spectrum of granulomatous-lymphocytic interstitial lung disease associated with Good's syndrome.

摘要

Good 综合征与胸腺瘤和获得性免疫缺陷相关。一位 54 岁男性因咳嗽就诊于我院。胸部影像学显示弥漫性结节影和前纵隔肿块。实验室检查发现低丙种球蛋白血症和 B 淋巴细胞计数减少。免疫球蛋白治疗后肺部结节明显消退。纵隔肿块和残余结节被手术切除,病理诊断分别为 AB 型胸腺瘤和伴有 T 淋巴细胞优势性肺泡炎的坏死性类上皮肉芽肿。这些病变的整体表现大多符合与 Good 综合征相关的肉芽肿性淋巴细胞性间质性肺病的谱。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d444/8545647/568181f8fad5/1349-7235-60-3137-g001.jpg

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