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Diagnostic Time Lag of Pediatric Haemophagocytic Lymphohistiocytosis and Patient Characteristics: A Retrospective Cohort Study
The difficulties and challenges of applying the HLH-2004 diagnostic criteria to early identification and diagnosis of haemophagocytic lymphohistiocytosis have been fully addressed in previous studies. However, the distribution of the diagnostic time lag of haemophagocytic lymphohistiocytosis and rel...
Autores principales: | , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2021
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8247774/ https://www.ncbi.nlm.nih.gov/pubmed/34222154 http://dx.doi.org/10.3389/fped.2021.692849 |
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author | Li, Xun Yan, Haipeng Xiao, Zhenghui Zhang, Xinping Huang, Jiaotian Xiang, Shi-Ting Zheng, Mincui Yao, Zhenya Zang, Ping Zhu, Desheng Li, Liping Lu, Xiulan |
author_facet | Li, Xun Yan, Haipeng Xiao, Zhenghui Zhang, Xinping Huang, Jiaotian Xiang, Shi-Ting Zheng, Mincui Yao, Zhenya Zang, Ping Zhu, Desheng Li, Liping Lu, Xiulan |
author_sort | Li, Xun |
collection | PubMed |
description | The difficulties and challenges of applying the HLH-2004 diagnostic criteria to early identification and diagnosis of haemophagocytic lymphohistiocytosis have been fully addressed in previous studies. However, the distribution of the diagnostic time lag of haemophagocytic lymphohistiocytosis and related patient characteristics remain unclear. This study investigated the time lags between symptom onset and diagnosis and between hospital admission and diagnosis among pediatric patients with haemophagocytic lymphohistiocytosis, and identified factors that associated with a shorter or longer diagnostic time lag. The cohort of patients with haemophagocytic lymphohistiocytosis was drawn from a tertiary children's hospital and consisted of 122 pediatric patients. The distributions of symptom-to-diagnosis and admission-to-diagnosis time lags were assessed. Clinical characteristics within 48 h of admission and the fulfillment of HLH-2004 diagnostic criteria were compared among admission-to-diagnosis time lag categories. Logistic regression analyses were conducted to identify factors associated with an admission-to-diagnosis time lag >3 days. The median interval from first symptom onset to HLH diagnosis was 12 days (range 4–71 days) and the median interval from hospital admission to HLH diagnosis was 2 days (range 0–23 days). The following factors were negatively associated with admission-to-diagnosis > 3 days: Epstein–Barr virus infection; admission through pediatric intensive care unit; diagnosis established without NK-cell activity and soluble CD25 tests; the performance of all readily available diagnostic tests for HLH (within 48 and 72 h); concurrent fever, splenomegaly, and cytopenias within 48 h; hemophagocytosis, hypertriglyceridemia and/or hypofibrinogenemia within 48 h; and elevated ferritin, total bilirubin, alanine aminotransferase, and prothrombin time within 48 h. Our findings suggest that performance of adequate diagnostic tests for HLH is essential for early diagnosis of HLH. Once suspected, immediate and adequate diagnostic tests for HLH should be arranged for PICU patients. Improvements in diagnostic procedures and monitoring plans are needed to promote early diagnosis of HLH. |
format | Online Article Text |
id | pubmed-8247774 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-82477742021-07-02 Diagnostic Time Lag of Pediatric Haemophagocytic Lymphohistiocytosis and Patient Characteristics: A Retrospective Cohort Study Li, Xun Yan, Haipeng Xiao, Zhenghui Zhang, Xinping Huang, Jiaotian Xiang, Shi-Ting Zheng, Mincui Yao, Zhenya Zang, Ping Zhu, Desheng Li, Liping Lu, Xiulan Front Pediatr Pediatrics The difficulties and challenges of applying the HLH-2004 diagnostic criteria to early identification and diagnosis of haemophagocytic lymphohistiocytosis have been fully addressed in previous studies. However, the distribution of the diagnostic time lag of haemophagocytic lymphohistiocytosis and related patient characteristics remain unclear. This study investigated the time lags between symptom onset and diagnosis and between hospital admission and diagnosis among pediatric patients with haemophagocytic lymphohistiocytosis, and identified factors that associated with a shorter or longer diagnostic time lag. The cohort of patients with haemophagocytic lymphohistiocytosis was drawn from a tertiary children's hospital and consisted of 122 pediatric patients. The distributions of symptom-to-diagnosis and admission-to-diagnosis time lags were assessed. Clinical characteristics within 48 h of admission and the fulfillment of HLH-2004 diagnostic criteria were compared among admission-to-diagnosis time lag categories. Logistic regression analyses were conducted to identify factors associated with an admission-to-diagnosis time lag >3 days. The median interval from first symptom onset to HLH diagnosis was 12 days (range 4–71 days) and the median interval from hospital admission to HLH diagnosis was 2 days (range 0–23 days). The following factors were negatively associated with admission-to-diagnosis > 3 days: Epstein–Barr virus infection; admission through pediatric intensive care unit; diagnosis established without NK-cell activity and soluble CD25 tests; the performance of all readily available diagnostic tests for HLH (within 48 and 72 h); concurrent fever, splenomegaly, and cytopenias within 48 h; hemophagocytosis, hypertriglyceridemia and/or hypofibrinogenemia within 48 h; and elevated ferritin, total bilirubin, alanine aminotransferase, and prothrombin time within 48 h. Our findings suggest that performance of adequate diagnostic tests for HLH is essential for early diagnosis of HLH. Once suspected, immediate and adequate diagnostic tests for HLH should be arranged for PICU patients. Improvements in diagnostic procedures and monitoring plans are needed to promote early diagnosis of HLH. Frontiers Media S.A. 2021-06-17 /pmc/articles/PMC8247774/ /pubmed/34222154 http://dx.doi.org/10.3389/fped.2021.692849 Text en Copyright © 2021 Li, Yan, Xiao, Zhang, Huang, Xiang, Zheng, Yao, Zang, Zhu, Li and Lu. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Pediatrics Li, Xun Yan, Haipeng Xiao, Zhenghui Zhang, Xinping Huang, Jiaotian Xiang, Shi-Ting Zheng, Mincui Yao, Zhenya Zang, Ping Zhu, Desheng Li, Liping Lu, Xiulan Diagnostic Time Lag of Pediatric Haemophagocytic Lymphohistiocytosis and Patient Characteristics: A Retrospective Cohort Study |
title | Diagnostic Time Lag of Pediatric Haemophagocytic Lymphohistiocytosis and Patient Characteristics: A Retrospective Cohort Study |
title_full | Diagnostic Time Lag of Pediatric Haemophagocytic Lymphohistiocytosis and Patient Characteristics: A Retrospective Cohort Study |
title_fullStr | Diagnostic Time Lag of Pediatric Haemophagocytic Lymphohistiocytosis and Patient Characteristics: A Retrospective Cohort Study |
title_full_unstemmed | Diagnostic Time Lag of Pediatric Haemophagocytic Lymphohistiocytosis and Patient Characteristics: A Retrospective Cohort Study |
title_short | Diagnostic Time Lag of Pediatric Haemophagocytic Lymphohistiocytosis and Patient Characteristics: A Retrospective Cohort Study |
title_sort | diagnostic time lag of pediatric haemophagocytic lymphohistiocytosis and patient characteristics: a retrospective cohort study |
topic | Pediatrics |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8247774/ https://www.ncbi.nlm.nih.gov/pubmed/34222154 http://dx.doi.org/10.3389/fped.2021.692849 |
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