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[一例弥漫性泛细支气管炎合并恶性胸腺瘤及干燥综合征]

[A case of diffuse panbronchiolitis complicated by malignant thymoma and Sjögren's syndrome].

作者信息

Okano A, Sato A, Suda T, Suda I, Yasuda K, Iwata M, Chida K

机构信息

Second Department of Internal Medicine, Hamamatsu University School of Medicine.

出版信息

Nihon Kyobu Shikkan Gakkai Zasshi. 1991 Feb;29(2):263-8.

PMID:2033900
Abstract

A patient with diffuse panbronchiolitis (DPB) complicated by malignant thymoma and Sjögren's syndrome with pseudolymphoma is reported. A 58-year-old woman developed productive cough, dyspnea and sicca in the mouth. Laboratory findings on admission indicated a high titer of cold hemagglutinin, positive of anti-DNA and negative PPD skin test. Chest-X ray revealed diffuse reticulonodular shadows in the lower lung field and anterior mediastinal mass shadow. Pulmonary function studies showed reduction of the vital capacity, forced expiratory volume in one second, DLco and hypoxia (PaO2; 69.1 Torr). Lung biopsy and resection were performed. Histologically infiltration of mononuclear cells and accumulation of foamy cells around respiratory bronchioles and stenosis of terminal bronchioles by granulation tissue was compatible with the diagnosis of DPB and the mediastinal tumor appeared to be a malignant thymoma invading to pericardium. Focal infiltration of lymphocytes was also recognized around the salivary glands (Sjögren's syndrome) and in the alveolar septa (pseudolymphoma). This case of DPB might have been associated with lymphoproliferative disorders.

摘要

报告了1例弥漫性泛细支气管炎(DPB)合并恶性胸腺瘤及伴有假性淋巴瘤的干燥综合征患者。一名58岁女性出现咳痰、呼吸困难和口腔干燥。入院时实验室检查发现冷凝集素滴度高、抗DNA阳性、PPD皮肤试验阴性。胸部X线显示下肺野弥漫性网状结节影及前纵隔肿块影。肺功能检查显示肺活量、一秒用力呼气容积、一氧化碳弥散量降低及低氧血症(动脉血氧分压;69.1 Torr)。进行了肺活检及切除术。组织学检查显示呼吸细支气管周围单核细胞浸润及泡沫细胞聚集,终末细支气管因肉芽组织而狭窄,符合DPB诊断,纵隔肿瘤似乎是侵犯心包的恶性胸腺瘤。在唾液腺周围(干燥综合征)及肺泡间隔(假性淋巴瘤)也发现了淋巴细胞灶性浸润。该DPB病例可能与淋巴增殖性疾病有关。

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