滤泡淋巴瘤合并副肿瘤性自身免疫多器官综合征一例报告并文献复习

Para-neoplastic autoimmune multi-organ syndrome associated with follicular lymphoma： a case report and literature review

目的 提高对副肿瘤性自身免疫多器官综合征（PAMS）的诊疗水平.方法 对1例滤泡淋巴瘤（FL）合并PAMS且肺受累患者的诊治过程进行回顾性分析,并通过文献复习总结PAMS患者的临床特征.结果 患者,女,49岁,主要临床症状为口腔、阴道溃疡,干咳、憋气.影像学提示多发淋巴结肿大.腹股沟淋巴结活检病理学检查提示为FL.应用糖皮质激素和沙利度胺治疗后口腔、阴道溃疡好转,呼吸困难进行性加重;给予R-CHOP（利妥昔单抗、环磷酰胺、表柔比星、长春地辛、泼尼松）方案6个疗程治疗后FL部分缓解,但呼吸困难无明显改善.结论 原因不明的口腔黏膜损害伴有与胸部影像学改变不相称的呼吸困难时,需高度怀疑PAMS,同时应积极寻找其伴发肿瘤.早期诊断并完整切除肿瘤是PAMS治疗关键.

Objective To broaden our knowledge of para-neoplastic autoimmune multi-organ syndrome （PAMS）.Methods 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.Results 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.Conclusion 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.