Li Xun, Yan Haipeng, Cai Zili, Li Xiao, Xie Longlong, Luo Ting, Wang Xiangyu, Yang Yufan, Gong Ling, Tang Minghui, Zhang Xinping, Huang Jiaotian, Lu Xiulan, Xiao Zhenghui
Pediatrics Research Institute of Hunan Province, Affiliated Children's Hospital of Xiangya School of Medicine, Central South University (Hunan Children's Hospital), Changsha, China.
Pediatric Intensive Care Unit, Affiliated Children's Hospital of Xiangya School of Medicine, Central South University (Hunan Children's Hospital), Changsha, China.
Ann Hematol. 2024 Dec;103(12):5145-5155. doi: 10.1007/s00277-024-06073-4. Epub 2024 Oct 27.
Understanding the early features and characteristics of hemophagocytic lymphohistiocytosis (HLH) is essential for identifying high-risk individuals and also providing valuable pathological insights. This study aims to investigate the characteristics and trends of blood and hepatic parameters before an HLH diagnosis was established. Longitudinal hematological and hepatic test results from pediatric patients with HLH and an age- and sex-matched control group were analyzed. According to the length of time between hospital admission and the establishment of the HLH diagnosis, the HLH cases were divided into early-onset (≤ 7 days) and late-onset (> 7days) groups. Among the 229 pediatric HLH patients, the length of time between hospital admission and the establishment of an HLH diagnosis ranged from 0 to 41 days (median = 4 days). Over 80% of pediatric HLH patients presented abnormal laboratory results for aspartate aminotransferase (AST), triglycerides, lactate dehydrogenase (LDH), and hemoglobin at admission. The abnormal rates in the initial platelet count, neutrophil count, and fibrinogen tests were 67.3%, 48.3%, and 52.2%, respectively. The initial test results for AST, alanine aminotransferase (ALT), LDH, serum sodium, and albumin showed AUCs > 80% for discriminating early-onset HLH. For the discrimination of late-onset HLH, the performance of initial test results was poor. To conclude, abnormalities in AST, triglycerides, LDH, and hemoglobin are early presentations of pediatric HLH; platelet, neutrophil, and fibrinogen levels may become abnormal at a relatively late stage of the HLH disease trajectory; and the initial test results for AST, ALT, LDH, serum sodium, and albumin can be used to identify suspected early-onset HLH.
了解噬血细胞性淋巴组织细胞增生症（HLH）的早期特征对于识别高危个体以及提供有价值的病理见解至关重要。本研究旨在调查在HLH诊断确立之前血液和肝脏参数的特征及变化趋势。分析了HLH儿科患者以及年龄和性别匹配的对照组的纵向血液学和肝脏检查结果。根据入院至HLH诊断确立之间的时间长度，将HLH病例分为早发型（≤7天）和晚发型（>7天）组。在229例儿科HLH患者中，入院至HLH诊断确立的时间长度为0至41天（中位数=4天）。超过80%的儿科HLH患者入院时天门冬氨酸氨基转移酶（AST）、甘油三酯、乳酸脱氢酶（LDH）和血红蛋白的实验室检查结果异常。初始血小板计数、中性粒细胞计数和纤维蛋白原检查的异常率分别为67.3%、48.3%和52.2%。AST、丙氨酸氨基转移酶（ALT）、LDH、血清钠和白蛋白的初始检查结果在鉴别早发型HLH时的曲线下面积（AUC）>80%。对于晚发型HLH的鉴别，初始检查结果的表现较差。总之，AST、甘油三酯、LDH和血红蛋白异常是儿科HLH的早期表现；血小板、中性粒细胞和纤维蛋白原水平可能在HLH病程的相对晚期变得异常；AST、ALT、LDH、血清钠和白蛋白的初始检查结果可用于识别疑似早发型HLH。
