Clinical profiles and risk factors of 7-day and 30-day mortality among 160 pediatric patients with hemophagocytic lympho
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(2020) 15:229
RESEARCH
Open Access
Clinical profiles and risk factors of 7-day and 30-day mortality among 160 pediatric patients with hemophagocytic lymphohistiocytosis Xun Li1†, Haipeng Yan2†, Xinping Zhang2, Jiaotian Huang2, Shi-Ting Xiang1, Zhenya Yao2, Ping Zang2, Desheng Zhu2, Zhenghui Xiao2* and Xiulan Lu2*
Abstract Background: Hemophagocytic lymphohistiocytosis (HLH) is a relatively rare and life-threatening disorder. Early mortality remains significantly high among patients with HLH. Our aim was to investigate clinical features and risk factors associated with 7-day and 30-day mortality among pediatric HLH patients. We retrospectively collected medical records of patients with discharge diagnosis of HLH between August 2014 and October 2018 from a tertiary children’s hospital in China. The main outcome measures were the 7-day and 30-day outcome after hospital admission. The associations between symptoms, concomitant diagnoses, laboratory test results, and the risk of 7-day and 30-day mortality were examined. Results: Among 160 pediatric HLH patients, 18 (11.3%) patients were deceased within 7 days after admission, and 46 (28.8%) patients were deceased within 30 days. The identified strong risk factors (OR > 10 and p < 0.05) for 30-day mortality were myocardial damage, severe pneumonia, respiratory failure, coagulopathy, gastrointestinal disorder, and multiple organ dysfunction syndrome (MODS). Factors strongly associated with 7-day mortality were sepsis, myocardial damage, shock, and respiratory failure. All patients deceased within 7 days developed hepatic dysfunction, coagulopathy, and MODS. Conclusions: The identified risk factors could help to stratify patients with high risk of early death, and need to be considered in the development of treatment protocols. As early mortality of HLH remains high, studies are needed to investigate how to initiate adequate HLH-directed treatment strategies for patients at higher risk of early death. Keywords: Child, Hemophagocytic lymphohistiocytosis, Hemophagocytic syndrome, Mortality, Risk factor
Background Hemophagocytic lymphohistiocytosis is a relatively rare and life-threatening disorder, characterized by extreme immune activation resulting in hypercytokinemia and immune mediated injury of multiple organ systems [1–3]. There are two forms of HLH: primary HLH, also known as familial * Correspondence: [email protected]; [email protected] † Xun Li and Haipeng Yan contributed equally and should be considered as co-first authors. 2 Department of Pediatric Intensive Care Unit, Hunan Children’s Hospital, University of South China, Changsha, China Full list of author information is available at the end of the article
haemophagocytic lymphohistiocytosis (FHL), and secondary HLH [1]. Patients with primary HLH have underlying genetic defects, and usually have a family history of HLH [1, 3]. Secondary HLH is often associated with viral infections, such as Epstein-Barr Virus (EBV), malignancy, lymphoma, or autoimmune or autoinflammatory diseases [1, 3]. Without timely d
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