大细胞淋巴瘤（LBCL-IRF4）是2017年修订的世界卫生组织（WHO）分类中的一种新实体，最初主要在儿童中报道。在鉴定一名79岁患者后，我们评估了IRF4重排在成人弥漫性大B细胞淋巴瘤（DLBCLs）中的检测频率，这些淋巴瘤必须根据IRF4的荧光原位杂交（FISH）结果重新分类为LBCL-IRF4。我们用FISH在238例根据2008年WHO分类诊断为DLBCL的淋巴瘤中研究IRF4重排的存在情况。除了索引患者外，在237名患者中又检测出另外5例IRF4重排（2%）。这五例IRF4重排淋巴瘤的免疫组化特征与先前的LBCL-IRF4报告一致。其中一例被认为代表滤泡性淋巴瘤的转化，而不是原发LBCL-IRF4。在2例LBCL-IRF4中发现了BCL6重排，排除了BCL2和MYC重排。患者表现为有限期的病变，其中三名患者涉及头颈部，另外两名患者涉及肺和甲状腺。这项研究表明，虽然罕见，但LBCL-IRF4也应考虑老年患者和头颈部以外的局部区域。本文受版权保护。版权所有。

Large B-cell lymphoma with IRF4 rearrangement (LBCL-IRF4) is a new entity in the 2017 revised World Health Organisation (WHO) Classification, that was initially mainly reported in children. After identification of a 79-year-old patient we assessed how often IRF4 rearrangements can be detected in adult diffuse large B-cell lymphomas (DLBCLs) which have to be reclassified to LBCL-IRF4 based on fluorescent in-situ hybridisation (FISH) for IRF4.With FISH, we studied the presence of IRF4 rearrangements in 238 lymphomas that were diagnosed as DLBCL according to the previous WHO classification of 2008.In addition to the index patient, an IRF4 rearrangement was detected in another 5 out of 237 patients (2%). The immunohistochemical profile of these five IRF4 rearranged lymphomas was consistent with previous reports of LBCL-IRF4. One case was recognized to represent transformation of follicular lymphoma rather than de novo LBCL-IRF4. BCL6 rearrangements were found in 2 cases of LBCL-IRF4, BCL2 and MYC rearrangements were excluded. Patients presented with limited stage disease with involvement of the head and neck in three patients, and involvement of the lung and thyroid in two others. This study shows that, although rare, LBCL-IRF4 should also be considered in older patients and at localisations other than the head and neck region.This article is protected by copyright. All rights reserved.