滤泡性淋巴瘤合并副肿瘤性天疱疮和闭塞性细支气管炎1例报告

1 病例资料 患者女,56 岁,因"口腔溃疡伴盗汗、体重减轻近1 个月、全身多发淋巴结肿大1 周"于2019 年8 月1 日入住海军军医大学(第二军医大学)长海医院血液科.患者于2019 年6 月初无明显诱因出现口腔黏膜溃疡,伴盗汗、体重减轻,无发热、皮疹、关节痛、胸闷、吞咽困难等不适.2019 年6 月21 日于外院就诊,予地塞米松片口服、碳酸氢钠溶液和康复新液漱口、开喉剑喷雾剂外用,口腔溃疡无好转.

Follicular lymphoma associated with paraneoplastic pemphigus and bronchiolitis obliterans: a case report

Objective To investigate the clinical characteristics, imaging manifestations, diagnosis and treatment of follicular lymphoma (FL) associated with paraneoplastic pemphigus (PNP) and bronchiolitis obliterans (BO), so as to broaden our knowledge of FL associated with PNP. Methods a case of FL with PNP and BO was treated in our department, and the related literature was reviewed. Results A 56-year-old female patient suffered from oral ulcer, night sweat and weight loss. PET-CT showed multiple lymphadenopathy in the whole body, especially in the retroperitoneum. Retroperitoneal biopsy revealed follicular lymphoma. FL gained near complete remission with three cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin liposome, vinorelbine, dexamethasone). Although oral ulcer went into remission after treatment, dyspnea gradually appeared in the course of treatment. FL associated with PNP and Bo was considered according to clinical symptoms, imaging and pathological biopsy. Respiratory failure still progressed after treatment with glucocorticoid and immunoglobulin. During bronchoalveolar lavage, the patient occurred tension pneumothorax and respiratory failure. Although closed thoracic drainage, tracheotomy noninvasive ventilator-assisted ventilation and anti-infective treatment actively were performed, the patient gets worse and eventually died from septic shock and respiratory failure. Conclusions PNP and Bo should be considered in patients with painful oral mucosa and unexplained dyspnea. Extensive work-up should be performed to find out potential tumor after diagnosis of PNP and BO. PNP was the first symptom, BO was the main cause of death. Chemotherapy, hormone, immunosuppressant and immunoglobulin are the main treatment. Lymphoma and PNP can be relieved by treatment, but BO is progressive aggravation, eventually leading to death.