Para-neoplastic autoimmune multi-organ syndrome associated with follicular lymphoma: a case report and literature review
10.3760/cma.j.issn.0253-2727.2016.10.008
- VernacularTitle: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
- Author:
Dan CHEN
1
;
Chengyu LIN
;
Xiao HAN
;
Bo CHEN
;
Zhaohui LU
;
Xiaoyan CHANG
;
Minghui DUAN
Author Information
1. 100730,中国医学科学院、北京协和医学院北京协和医院内科
- Keywords:
Pemphigus;
Lymphoma,follicular;
Bronchiolitis obliterans;
Para-neoplastic autoimmune multi-organ syndrome
- From:
Chinese Journal of Hematology
2016;37(12):1049-1053
- CountryChina
- Language:Chinese
-
Abstract:
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.
