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目的探讨儿童NK/T细胞淋巴瘤的临床和病理学特点。方法收集3例发生在儿童的NK/T细胞淋巴瘤,进行临床和病理组织学观察以及免疫组化、原位杂交和T细胞受体基因重排检测,并复习相关文献。结果3例儿童NK/T细胞淋巴瘤中男女之比2:1,分别累及咽部、鼻翼和鼻腔。其中1例发病前有蚊咬超敏反应,1例病程中伴发皮疹。肿瘤组织呈弥漫性分布,细胞小到中等大,小细胞胞质稀少,核略不规则,染色质致密;中等大细胞核染色质呈点状或颗粒状,有多个核仁,核分裂象易见。可见大量凝固性坏死灶,凋亡小体常见。肿瘤组织围绕血管生长并浸润血管壁,周围小血管常发生透明变性。瘤细胞间及坏死灶附近可见明显的炎细胞浸润。原位杂交EBV均(+);免疫组化CD56、perforin、GranB、CD45RO和CD43(+);T细胞受体基因重排(-)。蚊咬超敏者皮损组织学上呈现炎性改变,原位杂交淋巴细胞约10%EBV(+),CD56(-)。结论儿童NK/T细胞淋巴瘤是高度恶性的淋巴造血组织肿瘤,在发病前或病程中可出现蚊咬超敏反应或皮肤非特异性炎性皮疹。
Objective To investigate the clinical and pathological features of childhood NK / T cell lymphoma. Methods Three cases of NK / T cell lymphoma in children were collected for clinical and histopathological observation, as well as immunohistochemistry, in situ hybridization and T cell receptor gene rearrangement. The related literatures were reviewed. Results The ratio of male to female in 3 children with NK / T cell lymphoma was 2: 1, involving throat, nose and nasal cavity respectively. One case before the onset of mosquito bite hypersensitivity, 1 case accompanied by skin rashes. Tumor tissue showed diffuse distribution, the cells were small to medium large, sparse cell cytoplasm, slightly irregular nuclei, chromatin dense; medium large chromatin chromatin was dot or granular, with multiple nucleoli, mitotic easy to see . Visible a large number of coagulation necrosis, apoptotic body common. Tumor tissue grows around the blood vessels and infiltrates the blood vessel walls, often with transparent degeneration around small blood vessels. Neoplastic cells and necrotic lesions can be seen near the obvious infiltration of inflammatory cells. In situ hybridization of EBV (+); immunohistochemical CD56, perforin, GranB, CD45RO and CD43 (+); T cell receptor gene rearrangement (-). Inflammatory lesions were found histologically in the hypersensitive skin of mosquito bites. The in situ hybridized lymphocytes were about 10% EBV (+) and CD56 (-). Conclusion Childhood NK / T-cell lymphoma is a highly malignant lymphoid hematopoietic tumor with mosquito bite hypersensitivity or nonspecific inflammatory skin rashes before or during the course of the disease.