Childhood malignancy-associated hemophagocytic lymphohistiocytosis: a retrospective, single-center study of 44 patients.
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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 - γ, TNF - α, 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.