Hu Qun, Xu Liyu, Zhang Xiaoming, Wang Jie, Zhou Zizi
Department of Respiratory, Critical Care and Sleep Medicine, Xiang'an Hospital, Xiamen University, Xiamen.
Department of Pulmonary and Critical Care Medicine, Fuzhou First Hospital, Fujian Medical University, Fuzhou.
Medicine (Baltimore). 2020 Jun 26;99(26):e20822. doi: 10.1097/MD.0000000000020822.
Extranodal natural killer/T-cell lymphoma (ENKTL) - nasal type is an aggressive form of malignant non-Hodgkin lymphoma with a very poor prognosis. Especially primary pulmonary ENKTL is a relatively rare form of non-Hodgkin lymphoma. Until now, the prevalence of primary pulmonary ENKTL is unknown. Since 2001, only 18 cases of primary pulmonary ENKTL have been published, in addition to the 2 cases reported here.
We describe 2 cases of primary pulmonary ENKTL. Both patients were male non-smokers, aged 61 and 49 years. Their main clinical symptoms included cold-like symptoms and intermittent fever (39.3°C and 38.8°C) for some days (40 days and 3 weeks). Both patients had no relevant personal or family medical history.
The patients were initially misdiagnosed with community-acquired pneumonia. Primary pulmonary ENKTL was confirmed by immunohistochemical staining of computed tomography-guided transthoracic needle biopsy specimens. Both cases were positive for CD56, CD3, and in situ hybridization for Epstein-Barr virus-encoded small RNA, but negative for CD20.
Initially, both patients were treated inadequately with intravenous moxifloxacin administration (unknown dosage and 400 mg q.d) in their local hospitals. Once diagnosed with primary pulmonary ENKTL in our hospital, they received 3 cycles of chemotherapy with combined regimens of dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide (SMILE), and in the second patient, bone marrow transplantation was performed following the third chemotherapy cycle.
Clinical follow-up after the chemotherapy showed that the condition of the first patient progressively deteriorated. He died 2 months following the initial diagnosis. However, the presence of the hemophagocytic lymphohistocytosis gradually improved in the second patient during chemotherapy. Ultimately, the second patient died of acute transplant rejection 6 months after the initial diagnosis.
The diagnosis of ENKTL should be considered when patients present with fever and expansile consolidation of the lung not responding to antibiotics. The diagnosis depends on histopathology and immunophenotyping. Percutaneous transthoracic needle biopsy is a safe and effective biopsy method. Chemotherapy may improve the prognosis, but this should be confirmed by prospective multicenter studies.
结外自然杀伤/T细胞淋巴瘤（ENKTL）-鼻型是一种侵袭性恶性非霍奇金淋巴瘤，预后很差。尤其是原发性肺ENKTL是一种相对罕见的非霍奇金淋巴瘤形式。到目前为止，原发性肺ENKTL的患病率尚不清楚。自2001年以来，除本文报道的2例病例外，仅发表了18例原发性肺ENKTL病例。
我们描述2例原发性肺ENKTL病例。两名患者均为男性，不吸烟，年龄分别为61岁和49岁。他们的主要临床症状包括类似感冒的症状和持续数天（40天和3周）的间歇性发热（体温分别为39.3°C和38.8°C）。两名患者均无相关个人或家族病史。
患者最初被误诊为社区获得性肺炎。通过计算机断层扫描引导下经胸针吸活检标本的免疫组织化学染色确诊为原发性肺ENKTL。两例病例的CD56、CD3以及爱泼斯坦-巴尔病毒编码的小RNA原位杂交均呈阳性，但CD20呈阴性。
最初，两名患者在当地医院接受了静脉注射莫西沙星治疗（剂量不详，其中一名患者为每日400毫克），但治疗不充分。在我院确诊为原发性肺ENKTL后，他们接受了3个周期的化疗，化疗方案为地塞米松、甲氨蝶呤、异环磷酰胺、L-天冬酰胺酶和依托泊苷联合方案（SMILE），第二名患者在第三个化疗周期后进行了骨髓移植。
化疗后的临床随访显示，第一名患者的病情逐渐恶化。他在初次诊断后2个月死亡。然而，第二名患者在化疗期间噬血细胞性淋巴组织细胞增生症的情况逐渐改善。最终，第二名患者在初次诊断后6个月死于急性移植排斥反应。
当患者出现发热且肺部有扩展性实变且对抗生素无反应时，应考虑ENKTL的诊断。诊断依赖于组织病理学和免疫表型分析。经皮经胸针吸活检是一种安全有效的活检方法。化疗可能改善预后，但这需要前瞻性多中心研究予以证实。
