Department of Hematology, Japanese Red Cross Kyoto Daiichi Hospital, Kyoto, Japan.
Department of Dermatology, Japanese Red Cross Kyoto Daiichi Hospital, Kyoto, Japan; Department of Dermatology, Kyoto City Hospital, Kyoto, Japan.
Curr Probl Cancer. 2022 Apr;46(2):100813. doi: 10.1016/j.currproblcancer.2021.100813. Epub 2021 Nov 19.
We herein report the rare case of a 72-year-old female who presented with paraneoplastic pemphigus (PNP) and bronchiolitis obliterans (BO) associated with follicular lymphoma (FL), who was successfully treated with obinutuzumab (GA101; G) and bendamustine (B). The patient had severe erosive stomatitis and bilateral conjunctival hyperemia that persisted for more than 6 months. A huge mass was found in the abdominal cavity, and a biopsy revealed grade 1 FL (stage IV). Based on a lip biopsy result, the patient was diagnosed with PNP associated FL. The patient received bendamustine and obinutuzumab (BG) chemotherapy and FL and PNP responded very well, but BO was additionally associated during the course of BG. BO progressed without exacerbation as BG therapy progressed to a 2 year maintenance therapy with G, and combination of azithromycin, inhaled bronchodilator therapy, and corticosteroid. She was followed up at the outpatient department with no pulmonary function decline or FL and PNP recurrence. Our case suggests that BG could be a promising treatment option for PNP and BO.
我们在此报告一例罕见病例，一名 72 岁女性患有副肿瘤天疱疮 (PNP) 和闭塞性细支气管炎 (BO)，同时伴有滤泡性淋巴瘤 (FL)，她成功接受了奥滨尤珠单抗 (GA101; G) 和苯达莫司汀 (B) 治疗。患者有严重的糜烂性口炎和双侧结膜炎充血，持续时间超过 6 个月。在腹腔中发现巨大肿块，活检显示 1 级 FL（IV 期）。根据唇部活检结果，患者被诊断为伴有 FL 的 PNP。患者接受苯达莫司汀和奥滨尤珠单抗 (BG) 化疗，FL 和 PNP 反应非常好，但在 BG 治疗过程中还出现了 BO。随着 BG 治疗进展为 2 年 G 维持治疗，以及阿奇霉素、吸入性支气管扩张剂治疗和皮质类固醇的联合治疗，BO 没有恶化但进展。她在门诊接受随访，没有出现肺功能下降或 FL 和 PNP 复发。我们的病例表明，BG 可能是 PNP 和 BO 的一种有前途的治疗选择。
