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[结节病-淋巴瘤综合征患者机化性肺炎急性加重导致猝死]

[Acute exacerbation of organizing pneumonia leading to sudden death in a patient with sarcoidosis-lymphoma syndrome].

作者信息

Iizuka Kazuhide, Ando Jun, Masuda Azuchi, Ochiai Tomonori, Tomomasa Ran, Yasuda Hajime, Takaku Tomoiku, Ota Yasunori, Yao Takashi, Gotoh Akihiko, Komatsu Norio

机构信息

Department of Hematology, Juntendo University School of Medicine.

Department of Next Generation Hematological Laboratory Medicine, Juntendo University School of Medicine.

出版信息

Rinsho Ketsueki. 2017;58(8):927-932. doi: 10.11406/rinketsu.58.927.

Abstract

The subject was an 83-year-old female; she had a history of Propionibacterium acnes-related sarcoidosis at the age of 79 years. At diagnosis, she was treated with clarithromycin and achieved remission. Four years later, during a routine physical check-up, she presented with pulmonary opacities and swelling of multiple lymph nodes. A definitive diagnosis of lymphoma could not be made by inguinal lymph node biopsy. The patient's general condition was good, and we observed her clinical course. Oh the 56th day of her illness, she died suddenly. Autopsy revealed diffuse large B-cell lymphoma (DLBCL). Sarcoidosis-lymphoma syndrome (SLS) was diagnosed after sampling hyalinized nodules from both lungs. The cause of death was organizing pneumonia around an epithelioid granuloma and cor pulmonale. Organization markedly increased around the DLBCL. These findings might be associated with cor pulmonale. Although SLS often appears during chronic active sarcoidosis, sudden death is rare and there are few reports on SLS in Japan. We report this case along with a review of the literature.

摘要

该患者为一名83岁女性;她在79岁时曾患过与痤疮丙酸杆菌相关的结节病。诊断时,她接受了克拉霉素治疗并实现缓解。四年后,在一次常规体检中,她出现了肺部混浊以及多个淋巴结肿大。腹股沟淋巴结活检未能确诊淋巴瘤。患者一般状况良好,我们观察了她的临床病程。在患病第56天时,她突然死亡。尸检显示为弥漫性大B细胞淋巴瘤(DLBCL)。从双肺采集玻璃样变结节进行活检后,诊断为结节病-淋巴瘤综合征(SLS)。死亡原因是上皮样肉芽肿周围的机化性肺炎和肺心病。DLBCL周围的机化明显增加。这些发现可能与肺心病有关。虽然SLS常出现在慢性活动性结节病期间,但猝死罕见,且日本关于SLS的报道很少。我们报告此病例并对文献进行综述。

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