Chin Ein Wan, Saniasiaya Jeyasakthy, Hitam Shahrul, Hassan Eyzawiah, Kah Wai Ngan
Department of Otorhinolaryngology, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.
Department of Otorhinolaryngology, Hospital Ampang, Ampang, Selangor Malaysia.
Indian J Otolaryngol Head Neck Surg. 2024 Apr;76(2):2137-2140. doi: 10.1007/s12070-023-04466-x. Epub 2023 Dec 28.
Extranodal natural killer T-cell lymphoma, nasal type (ENKTCL), is a rare form of non-Hodgkin lymphoma that is strongly related to Epstein-Barr Virus (EBV) infection and commonly presents as "midline lethal granuloma." Herein, we report a middle-aged lady who presented with a two-week history of fever, sore throat and constitutional symptoms. Intraoral examination revealed a lacerated soft palate with an ulcerated uvula. A diagnosis of ENKTCL was confirmed through deep biopsies under general anaesthesia supplemented with a positive serum EBV genome. Unfortunately, she succumbed due to disease progression with left frontal brain metastasis with concurrent pulmonary tuberculosis before treatment was completed. The recommended treatment is multimodality with L-asparaginase-containing regimes chemotherapy in an advanced stage, relapsed, or refractory ENKTCL for better outcomes. The quantification of circulating plasma EBV deoxyribonucleic acid (DNA) is helpful as the baseline of tumour load and a biomarker for monitoring treatment response and prognostication. We advocate repeated and deeper core tissue biopsies.
结外鼻型自然杀伤T细胞淋巴瘤（ENKTCL）是一种罕见的非霍奇金淋巴瘤，与爱泼斯坦-巴尔病毒（EBV）感染密切相关，通常表现为“中线致死性肉芽肿”。在此，我们报告一位中年女性，她有两周的发热、咽痛和全身症状病史。口腔检查发现软腭撕裂伴悬雍垂溃疡。通过全身麻醉下的深部活检并结合血清EBV基因组阳性确诊为ENKTCL。不幸的是，她在治疗完成前因疾病进展伴左额叶脑转移并并发肺结核而死亡。对于晚期、复发或难治性ENKTCL，推荐的治疗方法是采用含L-天冬酰胺酶方案的多模式化疗，以获得更好的疗效。循环血浆EBV脱氧核糖核酸（DNA）的定量分析有助于作为肿瘤负荷的基线以及监测治疗反应和预后的生物标志物。我们主张重复进行更深入的核心组织活检。
