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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

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:...

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Detalles Bibliográficos
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Editorial office of Chinese Journal of Hematology 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7348497/
https://www.ncbi.nlm.nih.gov/pubmed/28088968
http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2016.12.008
Descripción
Sumario: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.