Department of Hematology, Kobe City Medical Center General Hospital, 2-1-1, Minatojima-Minamimachi, Chuo-ku, Kobe, 650-0047, Japan.
Department of Pathology, Kobe City Medical Center General Hospital, 2-1-1, Minatojima-Minamimachi, Chuo-ku, Kobe, 650-0047, Japan.
BMC Pulm Med. 2019 Feb 21;19(1):48. doi: 10.1186/s12890-019-0815-9.
Pulmonary non-Hodgkin lymphoma (NHL) is rare. The most frequent subtype of pulmonary NHL is low-grade B-cell lymphoma, such as lymphoma of mucosa-associated lymphoma tissue. Extranodal natural killer cell/T-cell lymphoma, nasal type (ENKL) is characterized by predominant extranodal involvement and association with Epstein-Barr virus (EBV). ENKL with massive lung involvement has been infrequently reported, and its prognosis is extremely poor.
A 20-year-old Japanese man presented with intermittent fever lasting for 2 months. Radiological imaging demonstrated multiple nodules of uneven shape and size in both lungs. Video-assisted thoracic surgical lung biopsy showed abnormal lymphocyte infiltration, which was positive for CD3, CD56, and perforin. In situ hybridization for EBV-encoded RNA was positive. From these findings, he was diagnosed with ENKL with lung involvement. The patient was successfully treated with intensive combinational chemotherapy followed by allogeneic cord blood transplantation. He has been alive with continuous complete remission for 1 year after diagnosis.
Although ENKL involving the lung has been reported to have dismal outcomes, our patient showed long-term survival after intensive chemotherapy and up-front allogeneic hematopoietic transplantation. The present case highlights the importance of early diagnosis as well as allogeneic transplantation.
肺非霍奇金淋巴瘤（NHL）较为罕见。肺 NHL 最常见的亚型为低级别 B 细胞淋巴瘤，如黏膜相关淋巴组织淋巴瘤。结外自然杀伤/T 细胞淋巴瘤，鼻型（ENKL）以主要结外累及和与 EBV 相关为特征。伴有大量肺部累及的 ENKL 很少见，其预后极差。
一名 20 岁的日本男性因间歇性发热持续 2 个月就诊。影像学检查显示双肺多个形状和大小不均匀的结节。电视辅助胸腔镜肺活检显示异常淋巴细胞浸润，CD3、CD56 和穿孔素阳性。原位杂交 EBV-编码 RNA 阳性。根据这些发现，他被诊断为伴有肺部受累的 ENKL。患者接受强化联合化疗后成功进行了异基因脐带血移植。诊断后 1 年，他持续完全缓解，至今仍存活。
尽管有报道称累及肺部的 ENKL 预后不良，但我们的患者在接受强化化疗和一线异基因造血移植后显示出长期生存。本病例强调了早期诊断和异基因移植的重要性。
