Risk factors of early death in pediatric hemophagocytic lymphohistocytosis: Retrospective cohort study

Abstract

BackgroundHemophagocytic lymphocytosis (HLH) is a rare life-threatening hyperinflammatory syndrome in which early mortality remains high in patients with HLH.MethodsWe retrospectively collected the medical records of all pediatric patients diagnosed with HLH at the West China Second Hospital of Sichuan University between January 2014 and December 2020. Collect demographic, laboratory, clinical, genetic profiles, treatment information and perform statistical analysis from records. Risk factors for death 30 days after admission were evaluated using a multivariable logistic regression model.ResultsA total of 110 pediatric HLH patients were enrolled. The median age of patients was 44 months (IQR 23-100.5) and 62 (56.4%) in males. The 30-day mortality rate for admission to this cohort was 34 (30.9%). Multivariate logistic regression analysis showed that heart failure (OR = 13.389, 95% CI, 1.671–107.256, p = 0.015) and hypoproteinemia (OR = 4.841, 95% CI, 1.282–18.288, p = 0.020) were associated with increased early mortality in children with HLH.ConclusionsThese identified risk factors may help clinicians stratify patients with HLH and develop targeted treatment strategies. More research is needed to explore the best treatment strategies for patients with HLH to reduce early mortality in patients with HLH.

Molecular characterization of emerging Echovirus 11 (E11) shed light on the recombinant origin of a variant associated with severe hepatitis in neonates

“…Since 2018, severe neonatal infections and mortality associated with genogroup D5 of E11 have been reported in Taiwan, and in the Hubei, and Guangdong provinces of China 23,24 . In addition, this scenario has also been described in a retrospective study in China reporting data from an E11 outbreak occurred in 2019 3 . In this report, severe infections defined as hepatic dysfunction or liver failure were observed in 30 out of 105 (28.6%) neonates 3 .…”

“…3 In this report, severe infections defined as hepatic dysfunction or liver failure were observed in 30 out of 105 (28.6%) neonates. 3 However, a recently commentary of this study raises a concern about the results of those and potential new studies, if genomic data will be used to draw conclusions on association of E11 and unexpected clinical picture. 25 The main medical needs emerged from a general point of view is the lack of notifiable status for enterovirus infections in many EU countries, thus there may be more cases than are currently known.…”

“…In addition, this scenario has also been described in a retrospective study in China reporting data from an E11 outbreak occurred in 2019 3 . In this report, severe infections defined as hepatic dysfunction or liver failure were observed in 30 out of 105 (28.6%) neonates 3 . However, a recently commentary of this study raises a concern about the results of those and potential new studies, if genomic data will be used to draw conclusions on association of E11 and unexpected clinical picture 25…”

“…An upsurge of severe neonatal cases and fatalities linked to a novel variant of Echovirus 11 (E11) was documented in France, Italy and China. [1][2][3] World Health Organization (WHO) has gained attention owing to its association with severe neonatal infections since 2022. 4,5 Other cases have been reported occurring in European countries such as Spain, Sweden, and UK.…”

Rapid whole‐genome sequencing leading to specific treatment for two infants with haemophagocytic lymphohistiocytosis due to Wolman disease

“…1 Mortality can be as high as 30%. 2 There are many triggers for HLH, including malignancy, infection, primary genetic causes and genetic conditions where HLH is a rare secondary manifestation. 1 We report two infants presenting with HLH diagnosed with Wolman disease (WD) via ultra-rapid whole-genome sequencing (urWGS), through the Acute Care Genomics study.…”