小儿原发性胃肠道非霍奇金淋巴瘤的临床病理分析  

A clinicopathological analysis of pediatric primary gastrointestinal iymphoma

作　　者：杨姣[1] 周扬莹[1] 祝晟[1] 张屹[1] 傅春燕[1] 陈晨[1] 周建华[1] 

机构地区：[1]中南大学湘雅医学院病理学系,湖南省长沙市410078

出　　处：《临床小儿外科杂志》2011年第3期193-195,共3页Journal of Clinical Pediatric Surgery

基　　金：2010年国家大学生创新陛实验计划立项（YA10038）

摘　　要：目的探讨小儿原发性胃肠道非霍奇金淋巴瘤（primary gastrointestinallymphoma,PGIL）的临床病理特征及免疫表型。方法对10例小儿PGIL采用sP法行CD45、CD3、CD3e、CD5、CD10、CD15、CD20、CD30、CD45R01CD56、CD68，CD99、Bcl-2、Bcl-6、ALK、CyelinD1、Mum-1、MPO、PAX-5、TdT、TIA-1、Ki-67及细胞角蛋白（CK）免疫组织化学染色，运用原位杂交检测Epstein—Barr病毒编码的早期RNA（EBER），按2008年WHO淋巴造血系统肿瘤分类标准进行分类。结果①10例瘤细胞均表达CIM5、Ki-67，无一例表达CK；10例均经病理、免疫组化及原位杂交检测等确诊为不同类型PGIL；②临床表现为腹痛、腹泻、腹部包块、发热、贫血和消瘦等。发生在小肠5例、回盲部2例、结肠1例、直肠2例。6例为溃疡型，4例为隆起型；③B细胞性NHL7例，其中伯基特淋巴瘤5例，B淋巴母细胞性淋巴瘤1例，弥漫性大B细胞淋巴瘤1例；T细胞NHL3例，其中NK／T细胞淋巴瘤1例，肠病相关T细胞淋巴瘤1例，ALK阳性间变性大细胞淋巴瘤1例。结论小儿PGIL临床症状无特异性，以B细胞淋巴瘤多见，T细胞淋巴瘤少见，预后差，须与其他胃肠道恶性肿瘤相鉴别。Objective To investigate the clinicopathologic features and immunophenotypes in Pediatric Primary Gastrointestinal lymphoma （PGIL）. Methods Clinicopathologic analysis; Immunohistochemical Stainings（SP method） for CD45, CD3, CD3e, CD5,CD10,CD15, CD20, CD30,CD45RO, CD56,CD 68,CD99, CyclinD1 ,ALK , Bc1-2, Bc1-6, Mum- 1, MPO, PAX-5, TdT, TIA- 1, Ki- 67 and Cytokeratin （CK） being applied to the routine paraffin sections of all the 10 cases; EBER was detected by in situ hybridization. All cases were reclassified according to the WHO classification of lymphoma in 2008 with a retrospective study. Results （1）All 10 cases of PGIL expressed CIM5, Ki -67, None showed positive staining for CK, 10 cases were diagnosed as primary gastrointestinal non-Hodgkins lymphoma with different types; （2）The main clinical symptom were abdominal pain, diarrhea, abdominal mass,fever ,weight loss and hematochezia. 5 in small intestine,2 in ileocecal junction, 1 in colon,2 in rectum. Grossly, 6 cases showed ulcer pattern, 4 polypoid pattern of intestinal wall. The prognosis for these patients showed very poor; （3）cases （70%） were diagnosed as B-cell lymphomas , including 5 cases of Burkitt＇s lymphoma , 1 case of B lymphoblastic lymphoma and 1 case of diffuse large B cell lymphoma ; 3 cases （30%） were diagnosed as T cell lymphomas , including 1 case of natural killer/T-cell lymphoma , 1 case of enteropathy associated T cell lymphoma and 1 case of ALK positive anaplastic large cell lymphoma. Conclusions PGIL has no specific clinical symptoms, B cell lymphoma is the most common type in pediatric PGIL, T cell lymphoma is rare. These patients with PGIL had a very poor prognosis and should be distinguished from other malignant tumors of gastrointestinal.

关 键 词：胃肠肿瘤 淋巴瘤 非霍奇金 免疫表型分型 病理学 临床 

分 类 号：R735[医药卫生—肿瘤]