Low total cholesterol predicts early death in children with hemophagocytic lymphohistiocytosis

作者全名:"Xiao, Li; Xu, Ximing; Zhang, Zhiling; Dou, Ying; Guan, Xianmin; Guo, Yuxia; Yu, Jie"

作者地址:"[Xiao, Li; Dou, Ying; Guan, Xianmin; Guo, Yuxia; Yu, Jie] Chongqing Med Univ, Childrens Hosp, Natl Clin Res Ctr Child Hlth & Disorders, Dept Hematol & Oncol,Minist Educ,Key Lab Child Dev, Chongqing, Peoples R China; [Xu, Ximing] Chongqing Med Univ, Childrens Hosp, Big Data Ctr Childrens Med Care, Chongqing, Peoples R China; [Zhang, Zhiling] Nankai Univ, Sch Stat & Data Sci, Tianjin, Peoples R China"

通信作者:"Yu, J (通讯作者),Chongqing Med Univ, Childrens Hosp, Natl Clin Res Ctr Child Hlth & Disorders, Dept Hematol & Oncol,Minist Educ,Key Lab Child Dev, Chongqing, Peoples R China."

来源:FRONTIERS IN PEDIATRICS

ESI学科分类:CLINICAL MEDICINE

WOS号:WOS:000918370100001

JCR分区:Q2

影响因子:2.1

年份:2023

卷号:10

期号: 

开始页: 

结束页: 

文献类型:Article

关键词:hemophagocytic lymphohistiocytosis; total cholesterol; early mortality; pediatric; risk factor; lipids

摘要:"BackgroundHemophagocytic lymphohistiocytosis (HLH) is a rapidly progressive and potentially life-threatening disorder. Identifying risk factors and timely adjustment of the given treatment regimens is critical to reducing the early mortality in HLH patients. Hypocholesterolemia has been reported to be associated with poor prognosis in a variety of critical illnesses. However, serum cholesterol is rarely studied in HLH patients, and its prognostic value is unclear. MethodsWe conducted a retrospective cohort study in National Clinical Research Center for Child Health and Disorders (Chongqing), identifying pediatric HLH patients (including genetically confirmed pHLH and not genetically confirmed pHLH) diagnosed with the HLH-2004 protocol and treated with immunochemotherapy between January 2008 and December 2020. The patients' blood lipid levels at initial diagnosis of HLH, including triglycerides (TG), total cholesterol (TC), high-density lipoprotein cholesterol (HDL-C), low-density lipoprotein-cholesterol (LDL-C), were reviewed based on electronic medical records. Both Cox and logistic regression models were used to estimate the effects of blood lipid indicators on early death (within 30 days after diagnosis). ResultsA total of 353 patients were enrolled in the study, with a median age at diagnosis of 45 months. The observed 30-day mortality rate was 19.05% (64/336, 17 were lost to follow-up) and Kaplan-Meier-estimated 3-year survival rate was 61.67% (95% CI, 56.27%-67.59%). DNA-targeted sequencing of HLH-related genes was performed in 173 (49.0%, 173/353) patients (not all patients with suspected pHLH underwent genetic testing), and 29 patients were diagnosed with genetically confirmed pHLH. Lipid panel was performed in 349 patients: 91.98% (321/349) had TG >= 1.80 mmol/L, 62.75%(219/349) had TG >= 3.00 mmol/L, 92.84% (324/349) had HDL-C <= 1.04 mmol/L, 58.74% (205/349) had LDL-C <= 1.30 mmol/L and 24.64% (86/349) had TC <= 3.11 mmol/L. TC <= 3.11 mmol/L and BUN >= 7.14 mmol/L were the independent risk factors for 30-day mortality [HR(95%CI): 2.85(1.46, 5.57) and 2.90(1.48, 5.68), respectively]. The presence of one of these risk factors increased the 30-day mortality rate by 6-fold [HR = 6.24, 95%CI: (3.18, 12.22)] and the presence of two risk factors by nearly 10-fold [HR = 9.98, 95%CI: (4.23, 23.56)] compared with the patients with no risk factors. ConclusionSevere derangement of lipoproteins is common in children with HLH, and decreased TC is an independent risk factor for early death. Hypocholesterolemia should be included as a biomarker during the diagnosis and management of HLH patients."

基金机构:2022 Research Projects of Chongqing Municipal Health and Family Planning Commission [2022WSJK005]; Chongqing Innovation Program for Returned Overseas Chinese Scholars [ex2021112]

基金资助正文:This work was supported by the 2022 Research Projects of Chongqing Municipal Health and Family Planning Commission (2022WSJK005) and Chongqing Innovation Program for Returned Overseas Chinese Scholars (ex2021112).