Yuan Chang-Tsu, Cheng Ann-Lii, Hou Hsin-An
Graduate Institute of Clinical Medicine, National Taiwan University College of Medicine, Taipei, Taiwan.
Department of Pathology, National Taiwan University Cancer Center, Taipei, Taiwan.
Virchows Arch. 2023 Mar;482(3):641-645. doi: 10.1007/s00428-022-03385-6. Epub 2022 Aug 19.
Effusion-based lymphomas arising from pleural cavities are mostly B cell lymphomas. Non-B cell origins are very rare. These non-B cell lymphomas are usually disseminated and aggressive, and their underlying genetic changes are indeterminate. Here, we reported the first case of primary effusion anaplastic large cell lymphoma (ALCL) with exclusive involvement of a single body cavity, indolent initial presentation, and IRF4/DUSP22 rearrangement. This 73-year-old man had been in his usual health until he presented with exertional dyspnea for 1 month. Physical examination and whole-body imaging indicated isolated left pleural effusion without lymphadenopathies or tumors. Thoracentesis revealed anaplastic large lymphoid cells that were CD30 + , CD3 - , CD8 + , TIA1 + , CD138 - , Epstein-Barr virus-encoded small RNA - , human herpesvirus 8 - , and ALK - . Fluorescence in situ hybridization exhibited IRF4/DUSP22 rearrangement. A primary effusion ALK-negative ALCL was diagnosed. There was no evident progression without chemotherapeutics until 4 months after the diagnosis. Our findings expanded the spectrum of effusion-based lymphoma. Recognition of this disease could prevent misdiagnosis and guide treatment strategies for patients.
起源于胸腔的积液性淋巴瘤大多是B细胞淋巴瘤。非B细胞起源非常罕见。这些非B细胞淋巴瘤通常呈播散性且侵袭性强，其潜在的基因改变尚不明确。在此，我们报告了首例原发性积液间变性大细胞淋巴瘤（ALCL），其仅累及单个体腔，起病时病情进展缓慢，且存在IRF4/DUSP22重排。这名73岁男性此前身体状况良好，直至出现劳力性呼吸困难1个月。体格检查和全身影像学检查显示仅有左侧胸腔积液，无淋巴结肿大或肿瘤。胸腔穿刺抽出的细胞显示为间变性大淋巴细胞，CD30阳性、CD3阴性、CD8阳性、TIA1阳性、CD138阴性、爱泼斯坦 - 巴尔病毒编码的小RNA阴性、人类疱疹病毒8阴性、ALK阴性。荧光原位杂交显示存在IRF4/DUSP22重排。诊断为原发性积液ALK阴性ALCL。在诊断后4个月内，未经化疗病情无明显进展。我们的发现扩展了积液性淋巴瘤的谱系。认识这种疾病有助于防止误诊并指导患者的治疗策略。
