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Significance of serum ferritin level in hemophagocytic lymphohistiocytosis diagnosis

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机构： [1]Beijing Key Laboratory of Pediatric Hematology Oncology, National Center for Children’s Health, Beijing Children’s Hospital, Capital Medical University, Beijing, China [2]National Key Discipline of Pediatrics (Capital Medical University), Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, China [3]Key Laboratory of Major Diseases in Children, Ministry of Education, National Center for Children’s Health, Beijing Children’s Hospital, Capital Medical University, Beijing, China [4]Hematology Oncology Center, National Center for Children’s Health, Beijing Children’s Hospital, Capital Medical University, Beijing, China [5]Department of medical laboratory, National Center for Children’s Health, Beijing Children’s Hospital, Capital Medical University, Beijing, China [6]Laboratory of Hematologic diseases, National Center for Children’s Health, Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, Beijing, China

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关键词： admission diagnosis hemophagocytic lymphohistiocytosis serum ferritin threshold

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Introduction The threshold of serum ferritin (SF) level listed in diagnostic guidelines for hemophagocytic lymphohistiocytosis (HLH) of HLH-2004 has a low specificity. The goal of this research was to evaluate the role of admission SF in HLH diagnostic procedure and to find an appropriate threshold for a HLH suspected cohort with fever. Methods All patients admitted to Beijing Children's Hospital during the period of September 1, 2015 and July 31, 2016 with fever and SF tested at admission were included in this study. The significance of SF in HLH diagnosis and its relationships with HLH-relevant clinical characteristics were studied. Results Among 357 patients, 39 HLH cases were diagnosed (24 EBV-related HLH, 13 unknown etiologies triggered HLH, and two familial HLH). The best cutoff value of admission SF was 934 ng/mL, with sensitivity, specificity, positive predictive values (PPV), and negative predictive values (NPV) being 87.2%, 88.4%, 47.9%, and 98.3%, respectively. Compared to 500 ng/mL, specificity and PPV of the new SF standard in HLH diagnose increased by 11.7% and 14.0%, which indicated improvements in diagnostic ability of "non-HLH" and in veracity of "HLH" identification. Among four HLH patients whose admission SF was between 500 ng/mL and 934 ng/mL, HLH diagnosis was guaranteed by other laboratory results in two patients; however, possible misdiagnosis was made in the rest two patients. Conclusion Elevated cutoff value of admission SF level seems to be more appropriate for distinguishing HLH in patients with fever. The exact cutoff value of SF level at diagnosis needs to be determined.

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出版当年[2018]版：

大类 | 4 区医学

小类 | 4 区血液学

最新[2023]版：

大类 | 4 区医学

小类 | 4 区血液学

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出版当年[2017]版：

Q3 HEMATOLOGY

最新[2023]版：

Q3 HEMATOLOGY

影响因子： 2.2 最新[2023版] 2.4 最新五年平均 1.919 出版当年[2017版] 1.904 出版当年五年平均 2.03 出版前一年[2016版] 2.073 出版后一年[2018版]

第一作者：

第一作者机构： [1]Beijing Key Laboratory of Pediatric Hematology Oncology, National Center for Children’s Health, Beijing Children’s Hospital, Capital Medical University, Beijing, China [2]National Key Discipline of Pediatrics (Capital Medical University), Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, China [3]Key Laboratory of Major Diseases in Children, Ministry of Education, National Center for Children’s Health, Beijing Children’s Hospital, Capital Medical University, Beijing, China [4]Hematology Oncology Center, National Center for Children’s Health, Beijing Children’s Hospital, Capital Medical University, Beijing, China

通讯作者：

通讯机构： [1]Beijing Key Laboratory of Pediatric Hematology Oncology, National Center for Children’s Health, Beijing Children’s Hospital, Capital Medical University, Beijing, China [2]National Key Discipline of Pediatrics (Capital Medical University), Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, China [3]Key Laboratory of Major Diseases in Children, Ministry of Education, National Center for Children’s Health, Beijing Children’s Hospital, Capital Medical University, Beijing, China [4]Hematology Oncology Center, National Center for Children’s Health, Beijing Children’s Hospital, Capital Medical University, Beijing, China [6]Laboratory of Hematologic diseases, National Center for Children’s Health, Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, Beijing, China [*1]Beijing Key Laboratory of Pediatric Hematology Oncology, National Center for Children’s Health, Beijing Children’s Hospital, Capital Medical University, Beijing 100045, China. [*2]Laboratory of Hematologic diseases, Beijing Pediatric Research Institute, National Center for Children’s Health, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.

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Significance of serum ferritin level in hemophagocytic lymphohistiocytosis diagnosis

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