儿童NK/T细胞淋巴瘤3例临床病理分析  

Juvenile NK/T cell lymphoma:a clinicopathologic study of 3 cases

作　　者：张正祥[1] 安晓静[1] 石群立[1] 周航波[1] 马恒辉[1] 周晓军[1] 

机构地区：[1]南京大学医学院临床学院南京军区南京总医院病理科

出　　处：《诊断病理学杂志》2009年第2期88-91,共4页Chinese Journal of Diagnostic Pathology

摘　　要：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 juvenile NK/T cell lymphoma. Methods Three cases of juvenile NK/T cell lymphoma were collected for clinical and histological observation, immunohistochemistry, in situ hybridization and TCR gene rearrangement detection. Literature was further reviewed. Results The sites of involvement included pharynx, nasal ala and nasal cavity, respectively, in the 3 cases with male to female ratio 2：1, one of which presented hyperscnsitivity to mosquito bites prior to lymphoma, and the other accompanied skin rash during the course. The diffuse pattern was seen in neoplastic fissures. The tumor cells were small to medium in size. Small cells were characterized by scarce cytophasm and irregular hyperchromatic nuclei. Medium cells were characterized by ovoid nuclei with stippled or granular chromatin and multiple nucleoli. Mitotic figures were easily seen. Coagulative necrosis and apoptotic body were common. The tumor cells showed angiocentricity and angioinvasion in some cases. Hyaline degeneration of small vessels always took place around the neoplastic cells. There may also be a prominent infiltration of inflammatory cells in the tissue. The neoplastic cells were positive for CD56, perforin, GranB, CD45RO, CD43. EBV in situ hybridization was positive in 3 cases. No TCR gene rearrangement was detected. The skin lesion of hypersensitivity to mosquito bites showed inflammatory changes, and about 10% lymphoid cells were EBV positive by in situ hybridization, but negative for CD56. Conclusion Juvenile NK/T cell lymphoma is a highly malignant lymphoid hematopoietic tumor. Hypersensitivity to mosquito bites or nonspecific inflammatory rashs may be present before and during lymphoma development.

关 键 词：儿童 NK/T细胞 淋巴瘤 蚊咬超敏 EBV 

分 类 号：R733.1[医药卫生—肿瘤]