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CELL INTELLIGENCE · 肿瘤细胞治疗研究
肿瘤细胞治疗研究
儿童恶性肿瘤相关噬血细胞性淋巴组织细胞增生症：一项 44 例患者的回顾性单中心研究
英文原题：Childhood malignancy-associated hemophagocytic lymphohistiocytosis: a retrospective, single-center study of 44 patients.
查看英文原题
Childhood malignancy-associated hemophagocytic lymphohistiocytosis: a retrospective, single-center study of 44 patients.
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研究概要
儿童 M-HLH 患者的预后较差。
中文摘要
目的
本回顾性研究旨在探讨儿童恶性肿瘤相关噬血细胞性淋巴组织细胞增多症（M-HLH）的临床特征、管理和预后。
方法
这是一项单中心回顾性队列研究，纳入2003年1月至2024年12月本中心诊断为M-HLH的儿童患者。评估临床特征、治疗方案、总体缓解率（ORR）和总生存期（OS），并对潜在因素进行单变量和多变量分析，以确定预后因素。
结果
44例患者确诊M-HLH时的中位年龄为6.63岁（范围0.33–15.58岁）。多数患者（79.5%）的HLH由肿瘤诱发。最常见的恶性肿瘤为淋巴瘤（56.8%），其中以T/NK细胞亚型为主（36.4%），其次为急性白血病（27.3%）和朗格汉斯细胞组织细胞增多症（15.9%）。IL-2R、IFN-γ、TNF-α、IL-6和IL-10均显著升高。3例患者检出HLH相关基因（LYST、UNC13D和XIAP）的致病变异。与恶性肿瘤化疗后发生HLH的患者相比，在肿瘤确诊时即发生HLH的患者年龄显著更大，血小板和白蛋白水平更低（P<0.05）。HLH诊断后4周及最终随访时的ORR分别为63.6%和68.2%。6个月、1年和2年OS率分别为79%、69%和67%。血清铁蛋白升高（>5000 ng/mL）、年龄>10岁、乳酸脱氢酶>500 U/L以及未达到完全缓解（CR）均与OS较低相关（P<0.05）。在肿瘤诱发HLH中，达到CR可显著改善OS（P<0.01），但在化疗诱发HLH中未见此关联。最终随访时未达到CR是预后不良的独立危险因素。
结论
儿童M-HLH患者预后较差。HLH缓解对M-HLH预后至关重要。儿童M-HLH需要个体化且强化的治疗方案。
展开英文摘要原文
PURPOSE
This retrospective study aimed to investigate the clinical characteristics, management, and prognosis of pediatric malignancy-associated hemophagocytic lymphohistiocytosis (M-HLH).
METHODS
This was a retrospective, single-center cohort study, pediatric patients diagnosed with M-HLH diagnosed from January 2003 to December 2024 in our center were enrolled. Clinical characteristics, treatment regimens, overall response rate (ORR), and overall survival (OS) were evaluated. Univariate and multivariate analyses of potential factors were performed to identify prognostic factors.
RESULTS
Of the 44 patients, the median age at M-HLH diagnosis was 6.63 years (range: 0.33-15.58). Most patients (79.5%) developed HLH induced by tumors. Lymphoma was the most common malignancy (56.8%), predominantly of the T/NK - cell subtype (36.4%), followed by acute leukemia (27.3%) and Langerhans cell histiocytosis (15.9%). Marked elevations were observed in IL - 2R, IFN - , TNF - , IL - 6, and IL - 10. Pathogenic variants in HLH - associated genes ( LYST , UNC13D , and XIAP) were identified in three cases. Patients with the HLH at malignancy diagnosis were significantly older and had lower platelet and albumin levels compared to those with HLH after malignancy chemotherapy( P < 0.05). The ORR at the 4 week HLH diagnosis and at the final follow-up was 63.6% and 68.2%, respectively. The 6-month, 1-year, and 2-year OS rates were 79%, 69%, and 67%, respectively. Elevated serum ferritin (> 5000 ng/mL), age (> 10 years), lactate dehydrogenase (> 500 U/L), and failure to achieve CR were all associated with lower OS ( P < 0.05). Achieving CR significantly enhanced OS in malignancy-induced HLH ( P < 0.01), but not in chemotherapy-induced HLH. Failure to achieve CR by the final follow-up was an independent risk factor for a poor prognosis.
CONCLUSIONS
The prognosis of pediatric M - HLH patients is poor. Remission of HLH is critical to the prognosis of M - HLH. Personalized and intensive treatment regimens are necessary for pediatric M - HLH.
论文信息
- 作者
- Huang C、Huang J、Le Q、Zhou Y
- 单位
- Emergency Department, West China Second University Hospital, Sichuan University, Chengdu, China.China
- 期刊
- Frontiers in immunology2026
- 原文标识
- PubMed 42183288 · DOI 10.3389/fimmu.2026.1801752
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