Pediatrics Research Institute of Hunan, Hunan Children's Hospital, Changsha, China.
Department of Pediatric Intensive Care Unit (PICU) and Hunan Provincial Key Laboratory of Emergency Medicine for Children, Hunan Children's Hospital, Changsha, China.
J Clin Immunol. 2023 Jul;43(5):989-998. doi: 10.1007/s10875-023-01458-y. Epub 2023 Mar 6.
The first step in diagnosing hemophagocytic lymphohistiocytosis (HLH) is to suspect its presence and then order the appropriate diagnostic tests. The development of screening procedures for HLH could facilitate early diagnosis. In this study, we evaluated the utility of fever, splenomegaly, and cytopenias as screening criteria for identifying pediatric HLH at an early stage, built a screening model using commonly measured laboratory parameters, and developed a step-wise screening procedure for pediatric HLH.
The medical records of 83,965 pediatric inpatients, including 160 patients with HLH, were collected retrospectively. The utility of fever, splenomegaly, hemoglobin level, and platelet and neutrophil counts at hospital admission as screening criteria for HLH was evaluated. For HLH patients who might be missed by screening based on the presence of fever, splenomegaly, and cytopenias, a screening model using common laboratory parameters was developed. Following that, a three-step screening procedure was then developed.
The criteria of cytopenias affecting two or more lineages plus fever or splenomegaly had a sensitivity of 51.9% and a specificity of 98.4% for identifying HLH in pediatric inpatients. Our screening score model comprises six parameters: splenomegaly, platelet count, neutrophil count, albumin level, total bile acid level, and lactate dehydrogenase level. The use of the validation set had a sensitivity of 87.0% and a specificity of 90.6%. A three-step screening procedure has been developed: Step 1: Is fever or splenomegaly present? (Yes: risk for HLH should be considered, go to Step 2; No: less likely HLH); Step 2: Are cytopenias affecting at least two lineages? (Yes: consider HLH; No: go to Step 3); Step 3: Calculate the screening score. Is the sum of the score greater than 37? (Yes: consider HLH; No: less likely HLH). The overall sensitivity and specificity of the three-step screening procedure were 91.9% and 94.4%, respectively.
A significant proportion of pediatric HLH patients present at the hospital without having all three symptoms: fever, splenomegaly, and cytopenias. Our three-step screening procedure, utilizing commonly available clinical and laboratory parameters, can effectively identify pediatric patients who may be at high risk for HLH.
诊断噬血细胞性淋巴组织细胞增生症（HLH）的第一步是怀疑其存在，然后进行适当的诊断测试。开发 HLH 的筛查程序可以促进早期诊断。本研究评估了发热、脾肿大和细胞减少作为识别儿科 HLH 的早期筛查标准的效用，建立了使用常用实验室参数的筛查模型，并为儿科 HLH 开发了逐步筛查程序。
回顾性收集 83965 例儿科住院患者的病历，包括 160 例 HLH 患者。评估入院时发热、脾肿大、血红蛋白水平以及血小板和中性粒细胞计数作为 HLH 筛查标准的效用。对于可能因发热、脾肿大和细胞减少而漏诊的 HLH 患者，建立了使用常见实验室参数的筛查模型。随后，开发了三步筛查程序。
影响两个或更多谱系的细胞减少加上发热或脾肿大的标准对识别儿科住院患者中的 HLH 具有 51.9%的敏感性和 98.4%的特异性。我们的筛查评分模型包含六个参数：脾肿大、血小板计数、中性粒细胞计数、白蛋白水平、总胆汁酸水平和乳酸脱氢酶水平。验证组的使用具有 87.0%的敏感性和 90.6%的特异性。已开发出三步筛查程序：步骤 1：是否存在发热或脾肿大？（是：应考虑 HLH 风险，进入步骤 2；否：不太可能 HLH）；步骤 2：是否有影响至少两个谱系的细胞减少？（是：考虑 HLH；否：进入步骤 3）；步骤 3：计算筛查评分。评分总和是否大于 37？（是：考虑 HLH；否：不太可能 HLH）。三步筛查程序的总体敏感性和特异性分别为 91.9%和 94.4%。
相当一部分儿科 HLH 患者在医院就诊时没有出现发热、脾肿大和细胞减少这三个症状。我们的三步筛查程序，利用常用的临床和实验室参数，可以有效地识别可能患有 HLH 的儿科患者。
