Uematsu Nozomu, Sumi Masahiko, Kaiume Hiroko, Takeda Wataru, Kirihara Takehiko, Ueki Toshimitsu, Hiroshima Yuki, Ueno Mayumi, Ichikawa Naoaki, Watanabe Masahide, Kobayashi Hikaru
Department of Hematology, Nagano Red Cross Hospital.
Department of Oncology, Nagano Red Cross Hospital.
Rinsho Ketsueki. 2018;59(1):69-74. doi: 10.11406/rinketsu.59.69.
A 59-year-old man who complained of abdominal pain was referred to our hospital. Computed tomography (CT) revealed mesenteric lymph node swelling and intestinal perforation. Histopathological study of the resected ileum and lymph node demonstrated diffuse proliferation of medium-sized atypical lymphocytes. Immunohistochemistry results were positive for cluster of differentiation (CD) 3, CD8, and CD56 cells, negative for CD5 and CD4 cells, and negative for Epstein-Barr virus-encoded RNA-fluorescent in situ hybridization (EBER-FISH). It also revealed the expression of γδ T-cell receptors. On the basis of these findings, enteropathy-associated T-cell lymphoma (EATL) was diagnosed. Although the patient received two courses of cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisone (CHOP) and dexamethasone, etoposide, ifosfamide, and carboplatin (DeVIC) therapy, facial nerve and lower limb paralysis manifested. Magnetic resonance imaging (MRI) and lumbar puncture revealed central nervous system invasion of the EATL. Despite intrathecal chemotherapy and high-dose cytarabine therapy, the patient's neurological symptoms deteriorated. Fluorodeoxyglucose positron emission tomography (FDG-PET) /CT scan showed the accumulation of FDG along both median and sciatic nerves, and he was diagnosed with neurolymphomatosis (NL). He died on day 120 after admission. Autopsy specimens exhibited infiltration of lymphoma cells in the median and sciatic nerves. Although only one case of suspected NL in a patient with type 2 EATL has been previously reported, we clinically diagnosed NL using FDG-PET/CT and confirmed the diagnosis by autopsy. This case is valuable in terms of the pathological diagnosis of NL.
一名59岁主诉腹痛的男性被转诊至我院。计算机断层扫描（CT）显示肠系膜淋巴结肿大和肠穿孔。对切除的回肠和淋巴结进行组织病理学研究，结果显示中等大小的非典型淋巴细胞弥漫性增殖。免疫组织化学结果显示，分化簇（CD）3、CD8和CD56细胞呈阳性，CD5和CD4细胞呈阴性，爱泼斯坦-巴尔病毒编码RNA荧光原位杂交（EBER-FISH）呈阴性。此外还显示了γδT细胞受体的表达。基于这些发现，诊断为肠病相关T细胞淋巴瘤（EATL）。尽管该患者接受了两个疗程的环磷酰胺、羟基柔红霉素、长春新碱和泼尼松（CHOP）以及地塞米松、依托泊苷、异环磷酰胺和卡铂（DeVIC）治疗，但仍出现了面神经和下肢麻痹。磁共振成像（MRI）和腰椎穿刺显示EATL侵犯中枢神经系统。尽管进行了鞘内化疗和大剂量阿糖胞苷治疗，患者的神经症状仍恶化。氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）/CT扫描显示FDG沿正中神经和坐骨神经积聚，他被诊断为神经淋巴瘤病（NL）。患者于入院后第120天死亡。尸检标本显示正中神经和坐骨神经有淋巴瘤细胞浸润。尽管此前仅报道过1例2型EATL患者疑似NL的病例，但我们通过FDG-PET/CT对NL进行了临床诊断，并通过尸检证实了诊断。该病例在NL的病理诊断方面具有重要价值。
