累及胰腺并以急性胰腺炎为表现的结外鼻型NK／T 细胞淋巴瘤一例报道及文献复习

摘要

目的：侵及胰腺的 NK／T 细胞淋巴瘤极为罕见，临床表现无特异性，容易被临床医生误诊或漏诊。该文拟提高对以急性胰腺炎为表现的结外鼻型 NK／T 细胞淋巴瘤的临床表现及诊治要点的认识。方法报道以急性胰腺炎为表现的结外鼻型 NK／T 细胞淋巴瘤1例，并以“NK／T Cell”及“Pancreas”、或“NK／T 细胞”及“胰腺”等为检索词在 Pubmed 数据库、中国期刊全文数据库（CNKI）、万方数据知识服务平台检索相关文献，收集资料完整的侵及胰腺的 NK／T 细胞淋巴瘤的病例，分析其临床特点、治疗方案及预后情况等。结果该例为38岁男性患者，因上腹痛及血清淀粉酶、脂肪酶升高收入我科。腹部 MRI 提示胰体部囊实性病变，行超声引导下胰腺穿刺提示恶性肿瘤细胞，倾向于 NK／T 淋巴瘤细胞。通过鼻咽部肿物的穿刺活检，最终确诊为结外鼻型 NK／T 细胞淋巴瘤。患者先后进行8个疗程化学治疗及2次放射治疗，胰腺病灶在6个疗程化学治疗后完全消失，鼻咽部病灶在6个疗程化学治疗后明显缩小，8个疗程化学治疗后几乎消失。检索文献后共收集国内外8例侵及胰腺的 NK／T 细胞淋巴瘤患者的资料，其中5例诊断为结外鼻型 NK／T 细胞淋巴瘤，3例原发于胰腺。共有7例有明确的随访资料，仅1例获得完全缓解，其余在诊断后8个月内死亡。结论NK／T 细胞淋巴瘤尽管罕见，临床医生应意识到其可作为急性胰腺炎的发病原因之一，影像学检查可发现病灶，确诊需依靠病理和免疫组织化学检测。治疗以化学治疗为主，辅以放射治疗。%Objective Natural killer T (NK/T)-cell lymphoma with pancreas involvement is ex-tremely rare with non-specific clinical manifestations,which is likely to be misdiagnosed or missed by clini-cians.This study aimed to deepen the understanding of clinical manifestations,diagnosis and treatment of ex-tranodal NK/T-cell lymphoma with pancreas involvement presenting as acute pancreatitis.Methods We re-ported one case of extranodal NK/T-cell lymphoma with pancreas involvement presenting as acute pancreatitis.Literature review was performed using the researching keywords of “NK/T Cell”and “Pancreas”in both Eng-lish and Chinese from PubMed,China national knowledge infrastructure (CNKI)and Wanfang databases.Clinical presentations,therapeutic regime and clinical prognosis of patients with NK/T-cell lymphoma with pan-creas involvement were retrospectively analyzed.Results A 38-year-old male was hospitalized due to epigas-tric pain coupled with elevated serum amylase and lipase levels.Abdominal magnetic resonance imaging (MRI)demonstrated a cystic and solid lesion in the pancreas.Ultrasound-guided percutaneous fine needle as-piration (FNA)from the pancreas revealed malignant cells,which were highly suggestive of lymphoma cells.The diagnosis of NK/T-cell lymphoma was finally confirmed by the biopsy of nasal cavity.He received 8 cycles of chemotherapy combined with 2 sessions of radiotherapy.The pancreatic lesions disappeared after 6 cycles of chemotherapy.The mass in the nasopharynx was shrank and almost disappeared after the 8 cycles of chemother-apy.Clinical data of 8 patients diagnosed with NK/T-cell lymphoma with pancreas involvement were retrieved.Five cases were diagnosed with extranodal NK/T-cell lymphoma with nasal type and the other 3 patients had primary involvement of the pancreas.Seven patients had explicit follow-up data.Only one case received com-plete remission and the remaining patients died within 8 months after diagnosis.Conclusions NK/T-cell lym-phoma with pancreas involvement is extremely rare.Clinicians should emphasize the possibility of NK/T-cell lymphoma as one of the causes of acute pancreatitis.Lesions can be identified by imaging examination.The di-agnosis depends on pathological examination and immunohistochemistry.Chemotherapy is the main treatment assisted by radiotherapy.