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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 investigate the clinicopathological, immunophenotypic and molecular biological features of sporadic Burkitt’s lymphoma (KB) in children and to raise awareness of the disease. Methods 3 cases of children with jaw sporadic cases of sporadic 2007-1-12 Department of Pathology, Peking University School of Medicine, Department of Pathology lymphoma archives and consultation cases. Combined with clinical features, the results of light microscope, immunophenotype, Epstein-Barr virus (EBV) in situ hybridization and interphase fluorescence in situ hybridization (FISH) were analyzed comprehensively. Results In 3 cases of BL, there were 2 males and 1 females, ranging in age from 5 to 11 years. Clinical manifestations of jaw tumor, swollen gums, loose teeth. Immunohistochemical staining showed that the tumor cells expressed CD20 and CD10, more than 95% of the tumor cells Ki-67 positive; in situ hybridization showed EBER1 & 2 negative; interphase FISH detection of c-myc gene rupture, including 1 case accompanied by IgH Gene breaks. Conclusion BL mostly occurs in children and adolescents and is a highly aggressive B-cell lymphoma. It is easy to involve the jaw. It is easy to be misdiagnosed as missed by clinical manifestations.