Lookzadeh Somayeh, Pourabdollah Toutkaboni Mihan, Jamaati Hamidreza, Rezaei Mitra, Marashian Mehran
Chronic Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Virology Research Center, NRITLD, Shahid Beheshti University of Medical Sciences,Tehran, Iran.
Tanaffos. 2020 Jan;19(1):74-78.
Extra-nasal types of Extra-nodal natural killer cell lymphoma (ENKL) have been known with poorer prognoses than nasal type with the worst responses to treatment. The current work introduces a case of ENKL with GI involvement with no nasal manifestations. We report a 56-year male farmer with fever, productive cough, dyspnea, anorexia, vomiting and chill in addition to malaise and cachexia of three months duration referred to a hospital with acute abdominal pain, and was diagnosed as peritonitis due to perforated terminal ileum ulcer before experiencing surgery as a case of acute abdomen. The pathologic study of the relevant biopsy showed "ulceration and necrosis with dense fibrinoleukocytic exudation and granulation tissue formation. CT scan determined a bilateral mass like haziness which was more likely to be metastatic. The review of the previous pathologic specimens raised Natural Killer/T cell Lymphoma (NKTL), the reason for which we focused on the patient's sinuses and nasal area as well as nasopharynx. There was no finding in examination and endoscopy of sinuses. Pathology also found malignant high grade non-Hodgkin T cell lymphoma in specimens obtained from debridement of ulcer at terminal ileum. It also showed that most of the tumor cells were positive for CD3, CD56, CD8, and LCA but negative for CD19, CD20 and AE1/AE3. Positive reactions for CD30 were shown by some cells. CD56, CD3, and CD8 were expressed by neoplastic cells and CD30 were positive in few cells. Proliferative activity (Ki67 index) was high (60-70%). This was the main base to diagnose an extra-nodal extra-nasal NK/T cell lymphoma. In conclusion, Intestinal changes at middle age, especially in men with nonspecific clinical manifestations is highly advised to be studied pathologically and genetically for T cell types like CD30 positive T cells which are usually engaged in ENKTL.
结外自然杀伤细胞淋巴瘤（ENKL）的鼻外型预后比鼻型差，对治疗反应最差。目前的研究介绍了一例累及胃肠道但无鼻部表现的ENKL病例。我们报告一名56岁男性农民，持续三个月出现发热、咳痰、呼吸困难、厌食、呕吐、寒战，伴有全身不适和恶病质，因急性腹痛入院，在作为急腹症接受手术前被诊断为末端回肠溃疡穿孔所致腹膜炎。相关活检的病理研究显示“溃疡和坏死，伴有致密的纤维蛋白白细胞渗出和肉芽组织形成”。CT扫描显示双侧有肿块样模糊影，更可能是转移性的。回顾之前的病理标本发现了自然杀伤/T细胞淋巴瘤（NKTL），因此我们重点检查了患者的鼻窦、鼻腔以及鼻咽部。鼻窦检查和内镜检查均未发现异常。病理检查还在末端回肠溃疡清创标本中发现了恶性高级别非霍奇金T细胞淋巴瘤。结果还显示，大多数肿瘤细胞CD3、CD56、CD8和LCA呈阳性，但CD19、CD20和AE1/AE3呈阴性。部分细胞CD30呈阳性反应。肿瘤细胞表达CD56、CD3和CD8，少数细胞CD30呈阳性。增殖活性（Ki67指数）较高（60 - 70%）。这是诊断结外鼻外型NK/T细胞淋巴瘤的主要依据。总之，强烈建议对中年人的肠道病变，尤其是具有非特异性临床表现的男性，进行病理和基因研究，以确定是否存在通常与ENKTL相关的T细胞类型，如CD30阳性T细胞。
