Department of Undergraduate Medical Education, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
Division of Hematology and Oncology, Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
Eur J Haematol. 2024 May;112(5):723-730. doi: 10.1111/ejh.14163. Epub 2023 Dec 28.
To describe cases of Kaposi's sarcoma-associated herpesvirus (KSHV)-associated multicentric Castleman's disease (MCD) and primary effusion lymphoma (PEL) in patients with HIV from a large, safety-net hospital system in Dallas, Texas, USA.
We conducted a retrospective review of patients with HIV-associated PEL and/or MCD.
Twelve patients with PEL and 10 patients with MCD were identified. All patients were male and 17 of 20 were men who have sex with men; 66.7% of PEL patients and 50% of MCD patients had concurrent KS at the time of diagnosis; 42% of patients with PEL and 20% of patients with MCD died during the follow-up period. We noted improved survival in our cohort compared to previous studies, particularly in our PEL patients with a median survival of 11.4 months compared to 3-6-month median survival historically. Median follow-up time for MCD patients was 17.5 months. This improved survival is despite suboptimal antiretroviral therapy (ART) adherence at diagnosis, with only 50% of patients on ART at the time of MCD/PEL diagnosis.
These data highlight the importance of early recognition of PEL and MCD, and the larger-scale efforts needed to better understand the pathogenetic drivers of clinical outcomes in patients affected by KSHV-related diseases.
描述美国德克萨斯州达拉斯市一家大型医疗保障体系中 HIV 感染者的卡波西肉瘤相关疱疹病毒(KSHV)相关多中心Castleman 病(MCD)和原发性渗出性淋巴瘤(PEL)病例。
我们对 HIV 相关 PEL 和/或 MCD 患者进行了回顾性研究。
共发现 12 例 PEL 患者和 10 例 MCD 患者。所有患者均为男性,20 例患者中有 17 例为男男性行为者;66.7%的 PEL 患者和 50%的 MCD 患者在诊断时同时患有卡波西肉瘤;42%的 PEL 患者和 20%的 MCD 患者在随访期间死亡。与以往的研究相比,我们的队列中观察到生存率的提高,特别是我们的 PEL 患者的中位生存期为 11.4 个月,而历史上的中位生存期为 3-6 个月。MCD 患者的中位随访时间为 17.5 个月。尽管在诊断时抗逆转录病毒治疗(ART)的依从性并不理想,只有 50%的患者在 MCD/PEL 诊断时接受了 ART,但仍取得了这种生存率的提高。
这些数据强调了早期识别 PEL 和 MCD 的重要性,以及需要开展更大规模的研究来更好地了解受 KSHV 相关疾病影响的患者的临床结局的发病机制驱动因素。