Abstract：
Large B-cell lymphoma with IRF4 rearrangement (LBCL-IRF4) is a rare lymphoid neoplasm, usually occurring in the pediatric/young-adult age. Despite this, subsets of cases occur in elderly patients and express CD5, possibly entering the differential diagnosis with adult aggressive lymphomas, such as blastoid/pleomorphic mantle cell lymphoma (MCL-B/P). To better characterize the clinical-pathological features and differential diagnosis of LBCL-IRF4, we conducted a multi-centric study on 12 cases, focusing on CD5, Cyclin D1, and SOX11 expression. While most cases had typical presentation, adult-to-elderly age at diagnosis and unusual anatomic locations were reported in 3/12 (25.0%) and 2/12 (16.7%) patients, respectively. Histologically, CD5 was positive in 4/12 (33.3%) cases, Cyclin D1 was invariably negative, and SOX11 was weakly/partially expressed in 1/12 (8.3%) case. In conclusion, LBCL-IRF4 can have unconventional clinical presentations that may challenge its recognition. Although CD5 is frequently expressed, negativity for Cyclin D1 and SOX11 contributes to the differential diagnosis with MCL-B/P.
摘要：
伴有IRF4重排的大B细胞淋巴瘤（LBCL-IRF4）是一种罕见的淋巴瘤，通常发生在儿童/年轻成人年龄。尽管如此,病例亚群发生在老年患者中并表达CD5，可能进入与成人侵袭性淋巴瘤的鉴别诊断，例如囊样/多形性套细胞淋巴瘤（MCL-B/P）。为了更好地表征LBCL-IRF4的临床病理特征和鉴别诊断，我们对12例患者进行了多中心研究。关注CD5、细胞周期蛋白D1和SOX11表达。虽然大多数案例都有典型的表现，在3/12（25.0％）和2/12（16.7％）患者中报告了诊断时的成人至老年人年龄和异常解剖位置，分别。组织学上,4/12(33.3%)例CD5阳性,细胞周期蛋白D1总是阴性的，SOX11在1/12(8.3%)病例中弱/部分表达。总之,LBCL-IRF4可能具有非常规的临床表现，可能会挑战其识别。虽然CD5经常表达，CyclinD1和SOX11的阴性有助于MCL-B/P的鉴别诊断。
