以胃肠道症状首发伴有皮肤表现的血管内NK／T细胞淋巴瘤一例

Intravascular NK/T-cell lymphoma firstly manifesting as gastrointestinal tract symptom and compli-cared by skin lesions： a case report

患者男，51岁，上腹部胀痛4个月，颈项、躯干、双大腿结节、斑块1个月。当地多家医院行胃肠道相关检查，并予对症处理，治疗效果不佳。腹部皮肤结节组织病理检查：真皮下层和皮下脂肪小叶内大量增生的小血管腔内填塞中等偏大异形淋巴样细胞；免疫组化标记：血管腔内异形细胞CD2（＋＋＋）、CD4（-）、CD8（-）、CD3e胞质（＋＋）、CD99（＋＋）、CD43（＋＋＋）、CD56（＋＋＋）、细胞毒颗粒相关蛋白（TIA一1）（＋＋）、穿孔素（＋＋）、EBER（＋＋＋）、CD20（-）、CD79a（-）、CD30（-）、细胞角蛋白（-）、S100（-）、CD68（-）、血管内皮细胞CD31（＋）、CD34（＋）。诊断：以胃肠道症状首发伴有皮肤表现的血管内NK／T细胞淋巴瘤。给予环磷酰胺、长春新碱、柔红霉素、地塞米松＋依托泊苷方案化疗，病情得到迅速控制，目前仍在随访中。

A 51-year-old man presented with a 4-month history of upper abdominal distending pain and 1-month history of cutaneous nodules and plaques on the neck, trunk and bilateral thighs. The patient underwent many gastrointestinal tract examinations in several local hospitals, and symptomatic treatment did not work. The biopsy of nodules on the abdomen revealed medium- to large-sized atypical lymphoid cells within numerous small vessels in lower dermis and subcutaneous fat tissue. Additionally, the atypical cells were pre- sent exclusively within vascular lumina. Immunohistochemical labeling showed the reactivity of neoplastic cells to CD2, CD99, CD3e, CD43, CD56, Epstein-Barr virus-encoded small nuclear RNAs （EBER）, and cytotoxic proteins such as T-cell intracellular antigen-1 （TIA-1） and perforin, but not to CD4, CD8, CD20, CD79a, CD30, cytokeratin （CK）, SIO0, or CD68. The endothelial cells lining the involved vessels exhibited the reactivity to CD31 and CD34. Based on the above findings, the patient was diagnosed with intravascular NK/T-cell lym- phoma firstly manifesting as gastrointestinal tract symptom and complicated by skin lesions. Following com- bined chemotherapy with cyclophosphamide, daunorubicin, vincristine, prednisone and etoposide, the patient experienced a quick and satisfactory recovery and the follow-up still continued.