Chen D, Lin C Y, Han X, Chen B, Lu Z H, Chang X Y, Duan M H
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.
Zhonghua Xue Ye Xue Za Zhi. 2016 Dec 14;37(12):1049-1053. doi: 10.3760/cma.j.issn.0253-2727.2016.12.008.
To broaden our knowledge of para-neoplastic autoimmune multi-organ syndrome (PAMS). A patient with PAMS associated with follicular lymphoma and bronchiolitis obliterans treated in our hospital was retrospectively analyzed and the clinical features of PAMS were reviewed. A 49-year-old female patient suffered from painful ulcers in the oral cavity and vagina, dry cough and dyspnea. Imaging examinations suggested multiple lymph node enlargements. Inguinal lymph node biopsy revealed follicular lymphoma. Although the oral and vaginal ulcers went into remission with glucocorticoid and thalidomide therapy and follicular lymphoma gained partial remission with six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone) chemotherapy, respiratory failure still progressed. PAMS should be considered in patients with unexplained oral mucosa ulcers and dyspnea, which didn't match with the chest image manifestations. Extensive work-up should be performed to find out the potential tumor after diagnosis of PAMS. Early diagnosis and complete removal of tumor were essential to PAMS treatment.
为拓宽我们对副肿瘤性自身免疫性多器官综合征（PAMS）的认识。对我院收治的1例合并滤泡性淋巴瘤和闭塞性细支气管炎的PAMS患者进行回顾性分析，并复习PAMS的临床特征。1例49岁女性患者，出现口腔和阴道疼痛性溃疡、干咳及呼吸困难。影像学检查提示多发淋巴结肿大。腹股沟淋巴结活检显示为滤泡性淋巴瘤。尽管经糖皮质激素和沙利度胺治疗后口腔和阴道溃疡缓解，滤泡性淋巴瘤经6周期R-CHOP（利妥昔单抗、环磷酰胺、多柔比星、长春新碱、泼尼松龙）化疗获得部分缓解，但呼吸衰竭仍进展。对于有无法解释的口腔黏膜溃疡和呼吸困难且与胸部影像表现不符的患者，应考虑PAMS。诊断PAMS后应进行全面检查以发现潜在肿瘤。早期诊断并彻底切除肿瘤对PAMS治疗至关重要。
