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[一例肺朗格汉斯细胞组织细胞增多症]

[A case of pulmonary Langerhans' cell histiocytosis].

作者信息

Sakai Kimiko, Ohtsuki Yuji, Hirasawa Yutaka, Hashimoto Akihide, Nakamura Kenji

机构信息

Department of Respiratory Medicine, Kita-ishikai Uchiyama Hospital, 275-1, Shiromawari, Uchiko-cho, Kita-gun, Ehime, 791-3310, Japan.

出版信息

Nihon Kokyuki Gakkai Zasshi. 2003 Sep;41(9):685-90.

Abstract

A 42-yr-old woman was referred to our hospital because of multiple small nodules in a chest radiograph. She had no symptoms such as dyspnea, cough or sputum. A chest CT revealed many centrilobular small nodules and thin-walled cysts with predominance in the peripheral area of the lungs. The specimens obtained by thoracoscopic surgery showed granulomas with scattered eosinophils and numerous Langerhans' cells. The Langerhans' cells were positive for both S-100 protein and CD1a. These findings are compatible with pulmonary Langerhans' cell histiocytosis (LCH). Since the granulomas showed no fibrotic changes, the LCH may have been in its early stages. However, there were clusters of lymphocytes and macrophages around the terminal and respiratory bronchioles, and cystic lesions without cellular infiltrates, in the specimens. The former histologic findings suggested respiratory bronchiolitis causing interstitial lung disease and the latter are indistinguishable from centrilobular emphysema. Therefore, these smoking-related diseases may have been superimposed on the LCH in this patient.

摘要

一名42岁女性因胸部X线片发现多个小结节而转诊至我院。她没有呼吸困难、咳嗽或咳痰等症状。胸部CT显示许多小叶中心性小结节和薄壁囊肿,主要分布在肺外周区域。经胸腔镜手术获取的标本显示肉芽肿,伴有散在的嗜酸性粒细胞和大量朗格汉斯细胞。朗格汉斯细胞的S-100蛋白和CD1a均呈阳性。这些发现符合肺朗格汉斯细胞组织细胞增多症(LCH)。由于肉芽肿未见纤维化改变,LCH可能处于早期阶段。然而,标本中终末细支气管和呼吸性细支气管周围有淋巴细胞和巨噬细胞聚集,以及无细胞浸润的囊性病变。前者的组织学表现提示呼吸性细支气管炎导致间质性肺疾病,后者与小叶中心性肺气肿难以区分。因此,这些与吸烟相关的疾病可能叠加在了该患者的LCH上。

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