Department of Hematology and Oncology, Okayama University Hospital, 2-5-1, Shikata-cho, Kita-ku, Okayama, 700-8558, Japan.
Department of Otorhinolaryngology, Okayama University Hospital, 2-5-1, Shikata-cho, Kita-ku, Okayama, 700-8558, Japan.
Int J Hematol. 2022 Jul;116(1):139-145. doi: 10.1007/s12185-022-03306-y. Epub 2022 Feb 14.
A 44-year-old Japanese man presented with fever and sore throat. He had a history of refractory chronic sinusitis that did not respond to several years of pharmacotherapy, and underwent endoscopic sinus surgery (ESS) 5 months prior to his presentation, but his symptoms persisted. A biopsy specimen was taken from the right nasal cavity, and extranodal natural killer/T-cell lymphoma, nasal type (ENKTL) was diagnosed. Two years after complete remission was achieved by chemoradiation therapy, he developed hemophagocytic lymphohistiocytosis (HLH) without recurrence of ENKTL. Epstein-Barr virus (EBV)-DNA copy number was relatively high and EBV-infected lymphocytes (CD8 + T cells) were detected in the peripheral blood. Pathological review of the biopsy specimens taken during ESS showed that CD8 + T cells with slightly atypia infiltrating the stroma were EBV positive. These findings suggested that the patient had underlying chronic active EBV infection (CAEBV) that caused the refractory chronic sinusitis, eventually developed into ENKTL, and also caused HLH. Clinicians should consider adult-onset CAEBV in the differential diagnosis of patients with refractory chronic sinusitis.
一位 44 岁的日本男性因发热和咽痛就诊。他有难治性慢性鼻窦炎病史，几年的药物治疗均无反应，并且在就诊前 5 个月接受了内镜鼻窦手术（ESS），但症状仍持续存在。从右侧鼻腔取活检标本，诊断为结外 NK/T 细胞淋巴瘤，鼻型（ENKTL）。在接受放化疗达到完全缓解 2 年后，他出现噬血细胞性淋巴组织细胞增生症（HLH），但无 ENKTL 复发。EB 病毒（EBV）-DNA 拷贝数相对较高，外周血中检测到 EBV 感染的淋巴细胞（CD8+T 细胞）。ESS 期间取的活检标本的病理复查显示，浸润基质的 CD8+T 细胞略有异型性，呈 EBV 阳性。这些发现提示患者存在潜在的慢性活动性 EBV 感染（CAEBV），导致难治性慢性鼻窦炎，最终发展为 ENKTL，并导致 HLH。临床医生应在难治性慢性鼻窦炎患者的鉴别诊断中考虑成人 CAEBV。
