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成人朗格汉斯细胞组织细胞增多症伴独立复发的肺部和肝脏病变,经依托泊苷成功治疗。

Adult Langerhans cell histiocytosis with independently relapsing lung and liver lesions that was successfully treated with etoposide.

作者信息

Konno Satoshi, Hizawa Nubuyuki, Betsuyaku Tomoko, Yasuo Masanori, Yamamoto Hiroshi, Koizumi Tomonobu, Nishimura Masaharu

机构信息

First Department of Medicine, Hokkaido University School of Medicine, Sapporo.

出版信息

Intern Med. 2007;46(15):1231-5. doi: 10.2169/internalmedicine.46.6433. Epub 2007 Aug 2.

DOI:10.2169/internalmedicine.46.6433
PMID:17675775
Abstract

A 35-year-old man initially presented with cough and fever. Computed tomography (CT) revealed diffuse small cysts in the lung, and multiple nodules in the liver. Lung and liver biopsies revealed that pathology was consistent with Langerhans cell histiocytosis. Lung shadows increased despite cessation of smoking, whereas the liver involvement improved. After initiating treatment with prednisolone, the chest CT findings improved. However, the liver nodules started to increase while tapering prednisolone. Intravenous etoposide was started, and the liver nodules decreased markedly. The difference in the clinical course between the lung and liver lesions might have been the result of differences in the clonality of these two organs.

摘要

一名35岁男性最初表现为咳嗽和发热。计算机断层扫描(CT)显示肺部有弥漫性小囊肿,肝脏有多个结节。肺和肝脏活检显示病理与朗格汉斯细胞组织细胞增多症相符。尽管戒烟,肺部阴影仍增加,而肝脏受累情况改善。开始使用泼尼松龙治疗后,胸部CT结果改善。然而,在逐渐减少泼尼松龙用量时,肝脏结节开始增加。开始静脉注射依托泊苷后,肝脏结节明显减少。肺和肝脏病变临床过程的差异可能是这两个器官克隆性差异的结果。

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Rare lung diseases III: pulmonary Langerhans' cell histiocytosis.罕见肺部疾病 III:肺朗格汉斯细胞组织细胞增生症。
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