【摘要】 目的 探讨伴IRF4重排的大B细胞淋巴瘤的临床病理学特点。方法 观察4例伴IRF4重排的大B细胞淋巴瘤的组织形态、分析临床及分子遗传学特点。结果 临床特点：4例患者，男性1例，女性3例，年龄3.5～14岁，平均年龄8.4岁。发病部位单侧扁桃体3例，鼻咽部1例。病理检查：肿瘤细胞不规则结节状生长，局部弥漫分布伴星空现象，胞核显著，分裂象易见，增殖活性高。免疫表型：均表达CD20、PAX5、Bcl-6和MUM1;Ki-67阳性指数均>75%。4例均显示IRF4基因断裂重排。2例患者临床为Ⅳ期，1例Ⅱ期，1例Ⅰ期。已随访4～42个月，未见肿瘤复发及进展。结论 伴IRF4重排的大B细胞淋巴瘤是一种具有独特临床病理表现的大B细胞淋巴瘤，FISH检测IRF4基因有助诊断，常规手术或化疗后，预后良好。更多还原

【Abstract】 Objective To investigate the clinical and pathological features of large B-cell lymphoma with IRF4 rearrangement. Methods The histomorphology, clinical and molecular genetic characteristics of 4 cases of large B-cell lymphoma with IRF4 rearrangement were observed. Results Among the 4 patients, 1 was male and 3 were female. The age ranged from 3.5 to 14 years, with an average age of 8.4 years. The initial lesion was located in the unilateral tonsil in 3 cases and the nasopharynx in 1 case. Pathological examination showed that tumor cells grew irregularly in a nodular pattern, with local diffuse distribution accompanied by starry sky phenomenon, prominent nuclei, visible mitotic figures, high proliferative activity. Immunophenotypically, all the tumor cells expressed CD20, PAX5, Bcl-6 and MUM1;Ki-67 positive index was >75%. All 4 cases showed IRF4 gene break rearrangement. Two patients were clinically stage Ⅳ, one was stage Ⅱ, and one was stage Ⅰ. The patients were followed up for 4 to 42 months, and no tumor recurrence or progression was found. Conclusion Large B-cell lymphoma with IRF4 rearrangement is a large B-cell lymphoma with unique clinicopathological features. FISH detection of IRF4 gene is helpful for diagnosis. After conventional surgery or chemotherapy, the prognosis is good.更多还原