中国呼吸与危重监护杂志

中国呼吸与危重监护杂志

淋巴瘤继发副肿瘤性天疱疮相关性闭塞性细支气管炎一例并文献复习

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目的 探讨淋巴瘤继发副肿瘤性天疱疮(PNP)相关性闭塞性细支气管炎(BO)临床表现、诊断及治疗。 方法 对 1 例淋巴瘤合并 PNP 继发 BO 患者的临床资料及通过万方医学网和 PubMed 数据库检索文献得到的病例报道的临床资料进行分析。 结果 患者女性,44 岁,主因“反复口腔溃疡 2 年,呼吸困难 14 个月”收入院。1 年前口腔溃疡考虑为 PNP。肺功能示严重的阻塞性通气功能障碍,支气管舒张试验阴性。胸部 CT 示双肺细支气管扩张。支气管肺泡灌洗液示中性粒细胞比例升高。B 超引导下右腹股沟淋巴结、颈部淋巴结穿刺,病理为非霍奇金淋巴瘤。结合临床、影像、病理结果考虑诊断为淋巴瘤继发 PNP 和 BO。予 CHOP 方案化疗 10 个周期,淋巴瘤和天疱疮缓解,但 BO 加重,死于呼吸衰竭。文献报道 15 例淋巴瘤继发 PNP 和 BO,60%(10/15)女性患者,47%(7/15)患者的原发病是滤泡性淋巴瘤,47%(7/15)患者没有获得病理组织学诊断 BO。淋巴瘤-PNP-BO 作为临床综合征,PNP 为首发表现的占 67%(10/15)。患者从发病到死亡的时间是 7~60 个月。 结论 BO 是继发淋巴瘤合并 PNP 的少见并发症,PNP 是大部分患者的首发表现,BO 是主要死亡原因。治疗以化疗、激素联合免疫抑制剂为主,淋巴瘤和 PNP 可缓解,但 BO 进行性加重。

Objective To explore the clinical characteristics, diagnose and treatment of bronchiolitis obliterans (BO) associated with lymphoma and paraneoplastic pemphigus (PNP). Methods One patient with BO associated with lymphoma and paraneoplastic pemphigus was presented and literatures which searched through Wanfang Med online and PubMed on this subject were reviewed. Results A 44-year-old woman was admitted to this hospital because of oral ulcer for two years, and persistent dyspnea for fourteen months. She had been diagnosed with PNP one year earlier. Pulmonary function displayed severe obstructive ventilator dysfunction, negative response to the bronchodilator. Chest CT showed bronchiolectasis. The ratio of neutrophils increased in bronchoalveolar lavage fluid. The pathology of ultrasound-guided neck and inguinal lymph node biopsy showed non-Hodgkin’s lymphoma. Based on the clinical presentation, the image and pathology of this patient, the diagnosis of having PNP with BO secondary to the lymphoma was made. After 10 cycles of CHOP chemotherapy, lymphoma and PNP went into remission, but the patient died from respiratory failure. The literatures were reviewed and 15 patients were found, out of them 60% (9/15) were female, 47% (7/15) follicular lymphoma and 47% (7/15) diagnosis of clinical BO which did not be supported by pathology. Lymphoma-PNP-BO was a clinical syndrome and 67% of the initial presentations were PNP. Patients died within 7 to 60 months. Conclusions BO associated with lymphoma and PNP is rare and the most common initial presentation is PNP. BO is the major cause of death. Treatments included chemotherapy, corticosteroid and immunosuppressor, lymphoma and PNP can be alleviated, but BO is aggravating.

关键词: 淋巴瘤; 副肿瘤性天疱疮; 闭塞性细支气管炎

Key words: Lymphoma; Paraneoplastic pemphigus; Bronchiolitis obliterans

引用本文: 张晓岩, 林江涛, 贾玉萍, 甘世保, 笪冀平, 于明安. 淋巴瘤继发副肿瘤性天疱疮相关性闭塞性细支气管炎一例并文献复习. 中国呼吸与危重监护杂志, 2019, 18(1): 70-75. doi: 10.7507/1671-6205.201709055 复制

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