Chen W, Gong Q X, Li X, Song G X, Wang Z, Xu W, Zhang Z H
Department of Pathology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China.
Department of Hematology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China.
Zhonghua Bing Li Xue Za Zhi. 2020 Oct 8;49(10):1003-1008. doi: 10.3760/cma.j.cn112151-20200601-00433.
To study the clinicopathological features of large B-cell lymphoma (LBCL) with IRF4 rearrangement. Seven cases of LBCL with IRF4 rearrangement collected at the First Affiliated Hospital of Nanjing Medical University from November 2018 to October 2019 were evaluated by hematoxylin and eosin staining, immunohistochemistry and fluorescence in situ hybridization detection. The relevant literature was reviewed. Four tumors were located in the tonsils, 2 tumors in the lymphoid nodes and one tumor in the adenoid.The patients were 3 males and 4 females patients with a median age of 24 years (range, 6 to 39 years).Microscopically, entirely follicular pattern was present in one case, entirely diffuse pattern in 2 cases, and follicular and diffuse pattern in other 4 cases. The tumor cells were medium to large in size and showed the morphology of centroblasts or blastoid cells with irregular nuclei, brisk mitotic activity in 3 cases and starry sky in 2 cases. All of the cases were positive for CD20, PAX-5, bcl-6, and MUM1 and had a Ki-67 index>80%, while CD10 and bcl-2 were positive in 3 cases. IRF4 gene rearrangement was identified in all cases and bcl-6 gene rearrangement in 2 cases. All patients presented with localized disease with clinical stage Ⅰ or Ⅱ, except one with stage Ⅳ at presentation and a new lesion in the mediastinum developed 8 months later. LBCL with IRF4 rearrangement is a clinicopathologically distinct entity. The observations reveal a broader spectrum of morphology and biological behaviors. The relationship between clinical stage and prognosis needs to be determined in more cases.
研究伴有IRF4重排的大B细胞淋巴瘤（LBCL）的临床病理特征。对2018年11月至2019年10月在南京医科大学第一附属医院收集的7例伴有IRF4重排的LBCL进行苏木精-伊红染色、免疫组织化学及荧光原位杂交检测，并复习相关文献。4例肿瘤位于扁桃体，2例位于淋巴结，1例位于腺样体。患者3例男性，4例女性，中位年龄24岁（范围6至39岁）。镜下，1例呈完全滤泡型，2例呈完全弥漫型，4例呈滤泡和弥漫混合型。肿瘤细胞中等至大，呈中心母细胞或母细胞样形态，核不规则，3例有活跃的有丝分裂象，2例有星空现象。所有病例CD20、PAX-5、bcl-6及MUM1均阳性，Ki-67指数>80%，3例CD10及bcl-2阳性。所有病例均检测到IRF4基因重排，2例检测到bcl-6基因重排。所有患者初诊时均为局限性疾病，临床分期为Ⅰ或Ⅱ期，仅1例初诊时为Ⅳ期，8个月后纵隔出现新病灶。伴有IRF4重排的LBCL是一种临床病理特征独特的实体。观察结果显示其形态学和生物学行为谱更广泛。临床分期与预后的关系需要更多病例来确定。
