Difficult Balance Between EBV Treatment and Posttransplant Immunosuppression: A Successful Transplant in a Child With Recurrent Epstein-Barr Virus–Induced Hemophagocytic Lymphohistiocytosis

Fałkowska, Anna; Prądzyńska, Katarzyna; Drabko, Katarzyna

doi:10.1016/j.transproceed.2021.03.044

Cited by 3 publications

(1 citation statement)

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“…Hemophagocytic lymphocytosis (HLH) is a rare hyperinflammatory syndrome characterized by an overactive immune system leading to hypercytokinemia and multi-organ dysfunction. HLH is divided into primary HLH (pHLH), which is due to genetic defects and usually familial, and secondary HLH (sHLH), which is due to infection (e.g., Epstein-Barr virus, EBV), tumors and other underlying factors (1)(2)(3)(4). Both can be detected at any age and can be lifethreatening if left untreated and effective (3,5).…”

Section: Introductionmentioning

confidence: 99%

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

Zhang

Dai

2022

Front. Pediatr.

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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.

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