Kim Hee-Jun, Lee Kyoungyul, Yoon Chang-Hwan, Bang Soo-Mee
Department of Internal Medicine, Chung-Ang University College of Medicine, Seoul Department of Pathology, Kangwon National University Hospital, Chuncheon, Gangwon-do Cardiovascular Center, Seoul National University Bundang Hospital, Seongnam, Gyeonggi-do Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Gyeonggi-do, Korea.
Medicine (Baltimore). 2017 Oct;96(43):e8010. doi: 10.1097/MD.0000000000008010.
Primary effusion lymphoma (PEL) is a rare disease of lymphomatous effusion in the body cavities in the absence of detectable mass and lymphadenopathy. PEL is predominantly related to the immunosuppressed patients infected with human herpes virus 8 (HHV-8). PEL-like lymphoma is negative for HHV-8 and human immunodeficiency virus (HIV) unlike PEL. The pathogenesis and prognosis of PEL-like lymphoma are unclear and there is no established treatment yet.
A 73-year-old male patient was admitted for evaluation of dyspnea on exertion with 1-week duration. His relevant examinations were completed.
PEL-like lymphoma was diagnosed.
The patient received chemotherapy including rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP), and palliative whole-brain radiotherapy, sequentially.
He died 3 months after the diagnosis.
Although the prognosis of PEL-like lymphoma may be better than PEL, our case showed poor disease course despite chemotherapy.
原发性渗出性淋巴瘤（PEL）是一种罕见的淋巴瘤，表现为体腔出现渗出液，而无可检测到的肿块和淋巴结病。PEL主要与感染人类疱疹病毒8（HHV-8）的免疫抑制患者有关。与PEL不同，PEL样淋巴瘤的HHV-8和人类免疫缺陷病毒（HIV）检测呈阴性。PEL样淋巴瘤的发病机制和预后尚不清楚，目前尚无既定的治疗方法。
一名73岁男性患者因劳力性呼吸困难持续1周入院评估。已完成相关检查。
诊断为PEL样淋巴瘤。
患者先后接受了包括利妥昔单抗、环磷酰胺、阿霉素、长春新碱和泼尼松龙（R-CHOP）的化疗以及姑息性全脑放疗。
诊断后3个月死亡。
尽管PEL样淋巴瘤的预后可能优于PEL，但我们的病例显示，尽管进行了化疗，病程仍不佳。
