Abstract：
To explore the clinical features, treatment, and prognosis of patients with lymphoma-associated hemophagocytic syndrome (LAHS) in a real-world clinical setting. We retrospectively examined LAHS patients diagnosed at our center between January 2016 and August 2023, focusing primarily on their clinical features, therapeutic approaches, overall response rate (ORR), and overall survival (OS). A combination of univariate and multivariate analyses was conducted to identify potential prognostic factors. A total of 86 patients diagnosed with LAHS were included to evaluate clinical characteristics and prognostic factors. Patients with T/NK cell lymphoma had a higher probability of developing hemophagocytic syndrome (HPS) during the clinical process than those with B cell lymphoma. The median survival time was 55 days for all patients, and 47 and 81 days for the T/NK cell LAHS and B cell LAHS cohorts, respectively (P = 0.025). Among the patients evaluated, the ORR was 42.2%. Patients starting with anti-lymphoma treatment had a better, albeit not significant, ORR than those beginning with anti-HPS treatment. In the univariate analysis, T/NK cell LAHS (P = 0.027), HPS onset at relapse (P = 0.036), higher baseline plasma EBV-DNA levels (> 4,000 copies/mL, P = 0.034), and treatments including cytokine adsorption and ruxolitinib (P < 0.001 and P = 0.017, respectively) were potentially associated with worse OS, while corticosteroid therapy benefited OS. In the multivariate analysis, T/NK cell LAHS (adjusted hazard ratio (aHR) = 2.007), cytokine adsorption therapy (aHR = 4.547), and corticosteroid therapy (aHR = 0.118) were independently associated with mortality. T/NK cell lymphoma was the main cause of LAHS and carried a worse prognosis. Whether anti-lymphoma or anti-HPS treatment should start first still requires prospective studies with larger sample sizes. The key point in controlling HPS is to block the cytokine storm promptly. Corticosteroid therapy is both effective and accessible and should be used early and in sufficient quantities.
摘要：
探讨其临床特点,治疗,淋巴瘤相关噬血细胞综合征（LAHS）患者在现实临床环境中的预后。我们回顾性检查了2016年1月至2023年8月在我们中心诊断的LAHS患者，主要关注他们的临床特征。治疗方法，总反应率（ORR），总生存率（OS）。进行单变量和多变量分析的组合以确定潜在的预后因素。共纳入86例诊断为LAHS的患者，以评估其临床特征和预后因素。T/NK细胞淋巴瘤患者在临床过程中发生噬血细胞综合征（HPS）的概率高于B细胞淋巴瘤患者。所有患者的中位生存时间为55天，T/NK细胞LAHS和B细胞LAHS队列的47天和81天，分别为(P=0.025)。在接受评估的患者中，ORR为42.2%。患者开始抗淋巴瘤治疗有一个更好的，尽管不重要，ORR比那些开始抗HPS治疗。在单变量分析中，T/NK细胞LAHS(P=0.027),HPS在复发时发作（P=0.036），较高的基线血浆EBV-DNA水平(>4,000拷贝/毫升，P=0.034),包括细胞因子吸附和鲁索替尼（分别为P<0.001和P=0.017）在内的治疗可能与OS恶化有关，而皮质类固醇治疗受益OS。在多变量分析中，T/NK细胞LAHS（调整后的危险比（AHR）=2.007），细胞因子吸附疗法（AHR=4.547），和皮质类固醇治疗(aHR=0.118)与死亡率独立相关.T/NK细胞淋巴瘤是LAHS的主要病因，预后较差。是否应首先开始抗淋巴瘤或抗HPS治疗仍需要具有更大样本量的前瞻性研究。控制HPS的关键是及时阻断细胞因子风暴。皮质类固醇治疗既有效又可获得，应及早使用并足够量。
