Histopathology Department, St James's Hospital, Dublin 8, Ireland.
Oncology Department, St James's Hospital, Dublin 8, Ireland.
Histopathology. 2024 Jan;84(2):399-401. doi: 10.1111/his.15079. Epub 2023 Oct 25.
Large B-cell lymphoma with IRF4 rearrangement (LBCL-IRF4) is a recently described entity included in the revised 4th edition of the WHO Classification of Haematolymphoid Tumours (2017). Here we highlight the difficulties in classification of those cases which arise in adult patients with unusual clinical features.
We present three cases with morphological and immunohistochemical features consistent with large B-cell lymphoma arising in adult patients, which were found to have isolated IRF4 rearrangements on FISH analysis. Each patient presented with advanced-stage disease and had a history of immunosuppression; clinical features that are not typical of LBCL-IRF4 and which make the distinction from DLBCL, not otherwise specified (NOS) challenging.
We propose that the clinical boundaries of LBCL-IRF4 arising in adult patients need further delineation to allow distinction from true cases of DLBCL, NOS.
伴有 IRF4 重排的大 B 细胞淋巴瘤（LBCL-IRF4）是最近描述的一种实体，被纳入修订版的 2017 年世界卫生组织血液淋巴肿瘤分类。在这里，我们强调了在具有不典型临床特征的成年患者中出现的那些病例在分类方面的困难。
我们展示了三例具有形态学和免疫组织化学特征的大 B 细胞淋巴瘤，这些肿瘤发生在成年患者中，经 FISH 分析发现存在孤立的 IRF4 重排。每位患者均表现为晚期疾病，且有免疫抑制病史；这些临床特征不典型，与非特指性弥漫性大 B 细胞淋巴瘤（DLBCL-NOS）的区分具有挑战性。
我们建议进一步划定成人 LBCL-IRF4 的临床界限，以区分真正的 DLBCL-NOS 病例。
