Lee Shin, Yamauchi Takahiro, Ishii Norito, Hashimoto Takashi, Kinoshita Keiichi, Imamura Shin, Kamiya Kenichi
Department of Hematology, Japan Red Cross Fukui Hospital, 2-4-1 Tsukimi, Fukui, Fukui, 918-8501, Japan.
Department of Hematology and Oncology, Faculty of Medical Sciences, University of Fukui, Fukui, Japan.
Int J Hematol. 2017 Dec;106(6):852-859. doi: 10.1007/s12185-017-2305-2. Epub 2017 Aug 8.
Paraneoplastic pemphigus (PNP) is a rare, fatal, paraneoplastic autoimmune mucocutaneous blistering disease, commonly associated with lymphoproliferative disorders, including malignant lymphomas. Lymphoproliferative disorders associated with PNP are sometimes associated with a serious lung complication, bronchiolitis obliterans (BO). Due to its rarity, guidelines for the management of PNP have not been established. Furthermore, most patients die within 1 year. Here we report the successful treatment of lymphoma-associated PNP and BO using R-CHOP chemotherapy. A 53-year-old Japanese man was admitted to our hospital for severe erosive stomatitis. Computed tomography and positron emission tomography showed multiple lymphadenopathies. He was diagnosed with follicular lymphoma (Ann Arbor stage IVA) and PNP-related BO. The patient underwent six cycles of R-CHOP and an additional cycle of rituximab. Both the erosive stomatitis and the obstructive lung disease persisted, but complete response of the follicular lymphoma was achieved. The patient survived 27 months after diagnosis. Although he died from progressive respiratory failure due to BO, we note that this patient achieved the longest survival of any reported case of PNP-related BO associated with a lymphoproliferative disorder. The present case suggests that intensive immunochemotherapy for underlying lymphoma may improve the prognosis in patients with PNP-related BO associated with lymphoma.
副肿瘤性天疱疮（PNP）是一种罕见的、致命的副肿瘤性自身免疫性黏膜皮肤水疱病，通常与包括恶性淋巴瘤在内的淋巴增殖性疾病相关。与PNP相关的淋巴增殖性疾病有时会伴有严重的肺部并发症——闭塞性细支气管炎（BO）。由于其罕见性，尚未制定PNP的管理指南。此外，大多数患者在1年内死亡。在此，我们报告使用R-CHOP化疗成功治疗淋巴瘤相关的PNP和BO的病例。一名53岁的日本男性因严重糜烂性口腔炎入住我院。计算机断层扫描和正电子发射断层扫描显示多处淋巴结肿大。他被诊断为滤泡性淋巴瘤（Ann Arbor分期IVA期）和PNP相关的BO。该患者接受了6个周期的R-CHOP化疗以及额外1个周期的利妥昔单抗治疗。糜烂性口腔炎和阻塞性肺病均持续存在，但滤泡性淋巴瘤达到了完全缓解。患者在诊断后存活了27个月。尽管他死于BO导致的进行性呼吸衰竭，但我们注意到该患者是所有报道的与淋巴增殖性疾病相关的PNP相关BO病例中存活时间最长的。本病例表明，对潜在淋巴瘤进行强化免疫化疗可能会改善与淋巴瘤相关的PNP相关BO患者的预后。
