Hu Zhihong, Pan Zenggang, Chen Weina, Shi Yang, Wang Wei, Yuan Ji, Wang Endi, Zhang Shanxiang, Kurt Habibe, Mai Brenda, Zhang Xiaohui, Liu Hui, Rios Adan A, Ma Hilary Y, Nguyen Nghia D, Medeiros L Jeffrey, Hu Shimin
Department of Pathology and Laboratory Medicine, The University of Texas Health Science Center at Houston, Houston, TX 77030, USA.
Department of Pathology, Yale University School of Medicine, New Haven, CT 06510, USA.
Cancers (Basel). 2021 Feb 19;13(4):878. doi: 10.3390/cancers13040878.
Primary effusion lymphoma (PEL) is a rare type of large B-cell lymphoma associated with human herpesvirus 8 (HHV8) infection. Patients with PEL usually present with an effusion, but occasionally with an extracavitary mass. In this study, we reported a cohort of 70 patients with PEL: 67 men and 3 women with a median age of 46 years (range 26-91). Of these, 56 (80%) patients had human immunodeficiency virus (HIV) infection, eight were HIV-negative, and six had unknown HIV status. Nineteen (27%) patients had Kaposi sarcoma. Thirty-five (50%) patients presented with effusion only, 27 (39%) had an extracavitary mass or masses only, and eight (11%) had both effusion and extracavitary disease. The lymphoma cells showed plasmablastic, immunoblastic, or anaplastic morphology. All 70 (100%) cases were positive for HHV8. Compared with effusion-only PEL, patients with extracavitary-only PEL were younger (median age, 42 vs. 52 years, = 0.001), more likely to be HIV-positive (88.9% vs. 68.6%, = 0.06) and EBV-positive (76.9% vs. 51.9%, = 0.06), and less often positive for CD45 (69.2% vs. 96.2%, = 0.01), EMA (26.7% vs. 100%, = 0.0005), and CD30 (60% vs. 81.5%, = 0.09). Of 52 (50%) patients with clinical follow-up, 26 died after a median follow-up time of 40.0 months (range 0-96), and the median overall survival was 42.5 months. The median OS for patients with effusion-only and with extracavitary-only PEL were 30.0 and 37.9 months, respectively ( = 0.34), and patients with extracavitary-only PEL had a lower mortality rate at the time of last follow-up (35% vs. 61.5%, = 0.07). The median OS for HIV-positive and HIV-negative patients were 42.5 and 6.8 months, respectively ( = 0.57), and they had a similar mortality rate of 50% at last follow-up. In conclusion, patients presenting with effusion-only versus extracavitary-only disease are associated with different clinicopathologic features. PEL is an aggressive lymphoma with a poor prognosis, regardless of extracavitary presentation or HIV status.
原发性渗出性淋巴瘤（PEL）是一种罕见的大B细胞淋巴瘤，与人类疱疹病毒8（HHV8）感染相关。PEL患者通常表现为渗出液，但偶尔也会出现腔外肿块。在本研究中，我们报告了一组70例PEL患者：67例男性和3例女性，中位年龄为46岁（范围26 - 91岁）。其中，56例（80%）患者感染了人类免疫缺陷病毒（HIV），8例为HIV阴性，6例HIV状态不明。19例（27%）患者患有卡波西肉瘤。35例（50%）患者仅表现为渗出液，27例（39%）仅有腔外肿块，8例（11%）既有渗出液又有腔外病变。淋巴瘤细胞表现为浆母细胞、免疫母细胞或间变性形态。所有70例（100%）病例HHV8均呈阳性。与仅表现为渗出液的PEL患者相比，仅表现为腔外病变的PEL患者更年轻（中位年龄，42岁对52岁，P = 0.001），更可能为HIV阳性（88.9%对68.6%，P = 0.06）和EBV阳性（76.9%对51.9%，P = 0.06），而CD45阳性率更低（69.2%对96.2%，P = 0.01），EMA阳性率更低（26.7%对100%，P = 0.0005），CD30阳性率更低（60%对81.5%，P = 0.09）。在52例（50%）有临床随访的患者中，26例在中位随访时间40.0个月（范围0 - 96个月）后死亡，中位总生存期为42.5个月。仅表现为渗出液和仅表现为腔外病变的PEL患者的中位总生存期分别为30.0个月和37.9个月（P = 0.34），仅表现为腔外病变的PEL患者在最后随访时死亡率更低（35%对61.5%，P = 0.07）。HIV阳性和HIV阴性患者的中位总生存期分别为42.5个月和6.8个月（P = 0.57），他们在最后随访时的死亡率相似，均为50%。总之，仅表现为渗出液与仅表现为腔外病变的患者具有不同的临床病理特征。PEL是一种侵袭性淋巴瘤，预后较差，无论是否有腔外表现或HIV状态如何。
