由胸膜腔引起的基于积液的淋巴瘤主要是B细胞淋巴瘤。非B细胞起源非常罕见。这些非B细胞淋巴瘤通常具有播散性和侵袭性,它们潜在的遗传变化是不确定的。这里,我们报道了首例原发性积液间变性大细胞淋巴瘤(ALCL),仅累及单个体腔,懒惰的初次陈述,和IRF4/DUSP22重排。这位73岁的男子一直处于正常健康状态,直到表现为劳力性呼吸困难1个月。体格检查及全身显像提示左侧胸腔积液,无淋巴结肿大或肿瘤。胸腔穿刺术显示间变性大淋巴样细胞为CD30+,CD3-,CD8+,TIA1+,CD138-,爱泼斯坦-巴尔病毒编码的小RNA-,人类疱疹病毒8-,和ALK-。荧光原位杂交显示IRF4/DUSP22重排。诊断为原发性积液ALK阴性ALCL。直到诊断后4个月,没有化疗药物没有明显的进展。我们的发现扩大了基于积液的淋巴瘤的范围。认识到这种疾病可以防止误诊并指导患者的治疗策略。
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.