儿童颌部Burkitt’s淋巴瘤临床病理分析  

Clinical and pathologic features of jaw Burkitt's lymphoma in children

作　　者：黄远洁[1] 郑杰[1] 刘翠苓[1] 宫丽平[2] 李敏[1] 黄欣[1] 董格红[1] 尹文娟[1] 周春菊[3] 张永红[3] 杜鹃[1] 高子芬[1] 

机构地区：[1]北京大学基础医学院病理学系,北京100191 [2]首都医科大学病理学系,北京100069 [3]首都医科大学附属北京儿童医院,北京100050

出　　处：《中国实用儿科杂志》2009年第9期708-710,共3页Chinese Journal of Practical Pediatrics

基　　金：首都医学发展科研基金(2007-1003)

摘　　要：目的探讨儿童颌部散发性Burkitt’s淋巴瘤(BL)的临床病理学、免疫表型及分子生物学特点,提高对该疾病的认识。方法3例儿童颌部散发性BL病例为2007年1—12月北京大学基础医学院病理学系淋巴瘤研究室存档及会诊病例。结合临床特征,对患儿光镜、免疫表型、EB病毒(EBV)原位杂交及间期荧光原位杂交(FISH)的检测结果进行综合分析。结果3例BL中,男性2例,女性1例;年龄5～11岁。临床均表现为颌部肿物,牙龈肿痛,牙齿松动。免疫组织化学染色显示肿瘤细胞表达CD20和CD10,大于95%以上的肿瘤细胞Ki-67阳性;原位杂交显示EBER1&2阴性;间期FISH检测均存在c-myc基因断裂,其中1例同时伴有IgH基因断裂。结论BL多发生在儿童及青少年,属高度侵袭性B细胞淋巴瘤,容易累及颌骨,与换牙临床表现相似而易被漏诊误诊。Objective To study the clinical, pathologic, immunohistoehemieal and molecular features of the sporadic Burkitt＇ s lymphoma（BL） involving the jaw in children in order to evaluate the role of immunohistochemical and molecular study in diagnosis of this entity and make a comprehensive understanding of this neoplasm. Methods Three cases of sporadic BL were retrospectively studied by analyzing their clinical, light microscopic and immunohistochemieal features, EBV infection detected by in situ hybridization, chromosomal break of c-mye and IgH genes by inter-phase fluo- rescence in situ hybridization （FISH）. Results All the 3 cases of sporadic BL occurred in children （5- 11y） including 2 males and 1 female presented with tumors involving the jaw with hyperemia and hemorrhage of the gingival and loosening of the teeth. Microscopically, the medium-sized tumor cells were monomorphic and proliferated in a diffuse pattern showing “starry-sky”. Immunohistochemieally, the tumor cells were positive for CD20 and CD10, over 95% positive for Ki-67. No EBV infection was found by in situ hybridization. Interphase FISH analysis detected e-myc gene break or amplification in all cases and 1 case with IgH gene break as well. Conclusion Sporadic BL is a high-grade malignant B cell lymphoma. Most affected patients were children and often the jaw is involved, which is similar to the dental transitional behavior. Molecular and immunohistochemical analysis can reduce the incidence of misdiagnosis and thus plays a vital role in its correct diagnosis and appropriate therapy.

关 键 词：淋巴瘤 免疫组织化学 荧光原位杂交 

分 类 号：R72[医药卫生—儿科]