Hemophagocytic Lymphohistiocytosis in the Medical ICU: A Single-Institution Cohort Study on Acute Liver Failure and Mortality

dc.contributor.authorAl Nasrallah, Nawar
dc.contributor.authorAl-Hader, Ahmad
dc.contributor.authorSamala, Niharika
dc.contributor.authorSears, Catherine R.
dc.contributor.departmentMedicine, School of Medicineen_US
dc.date.accessioned2021-02-12T21:46:19Z
dc.date.available2021-02-12T21:46:19Z
dc.date.issued2021-01-08
dc.description.abstractHemophagocytic lymphohistiocytosis is a life-threatening hyperinflammatory disorder that is associated with high morbidity and mortality in the ICU. It has also been associated with acute liver failure. Design: Retrospective observational study. Setting: Tertiary-care medical ICU. Patients: Thirty-one patients critically ill with hemophagocytic lymphohistiocytosis. Interventions: None. Measurements and main results: We performed a comprehensive review of critically ill hemophagocytic lymphohistiocytosis patients admitted to a tertiary-care medical ICU from January 2012 to December 2018. Most patients presented with constitutional symptoms and elevated liver enzymes and thrombocytopenia were common upon hospital admission. ICU admission laboratory and clinical variables were used to calculate Acute Physiology and Chronic Health Evaluation II, hemophagocytic syndrome diagnostic score, and model for end-stage liver disease. Mean age of the cohort was 48.1 years, and 45% were male. The mortality rate was 65% at 28 days and 77% at 1 year. About 28-day survivors were younger, had lower mean Acute Physiology and Chronic Health Evaluation II score (16.5 vs 23.0; p = 0.004), and higher mean hemophagocytic syndrome diagnostic score (249.1 vs 226.0; p = 0.032) compared with nonsurvivors. Survivors were less likely to receive mechanical ventilation, renal replacement therapy, or vasopressor support and were more likely to receive chemotherapy for hemophagocytic lymphohistiocytosis. In this ICU cohort, 29% were diagnosed with acute liver failure, of whom only 22% developed acute liver failure early during their hospital stay. Acute liver failure was associated with a higher model for end-stage liver disease score upon hospital admission. Available histology in those that developed acute liver failure showed massive hepatic necrosis, or histiocytic or lymphocytic infiltrates. Conclusions: Patients admitted to the ICU with hemophagocytic lymphohistiocytosis have a high mortality. Those who survived had lower Acute Physiology and Chronic Health Evaluation scores, had higher hemophagocytic syndrome diagnostic scores, are more likely to receive hemophagocytic lymphohistiocytosis specific chemotherapy, and are less likely to have organ failure. Hemophagocytic lymphohistiocytosis can be associated with acute liver failure especially when model for end-stage liver disease score is elevated upon admission.en_US
dc.description.sponsorshipSupported, in part, through T32HL091816 to Indiana University (Dr. Al Nasrallah). Dr. Sears receives research support to the institution through the American Cancer Society (128511-MRSG-15-163-01-DMC), National Institutes of Health, and Veterans Affairs Health Services, and is on the Scientific and Medical Advisory Boards for Biodesix and bioAffinity Technologies. The remaining authors have disclosed that they do not have any potential conflicts of interest.en_US
dc.eprint.versionFinal published versionen_US
dc.identifier.citationAl Nasrallah, N., Al-Hader, A., Samala, N., & Sears, C. R. (2021). Hemophagocytic Lymphohistiocytosis in the Medical ICU: A Single-Institution Cohort Study on Acute Liver Failure and Mortality. Critical Care Explorations, 3(1). https://doi.org/10.1097/CCE.0000000000000318en_US
dc.identifier.issn2639-8028en_US
dc.identifier.urihttps://hdl.handle.net/1805/25219
dc.language.isoen_USen_US
dc.publisherWolters Kluweren_US
dc.relation.isversionof10.1097/CCE.0000000000000318en_US
dc.relation.journalCritical Care Explorationsen_US
dc.rightsIUPUI Open Access Policyen_US
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 International*
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/*
dc.sourcePublisheren_US
dc.subjectCOVID-19en_US
dc.subjectObservational Studyen_US
dc.subjectHemophagocytic Lymphohistiocytosis (HLH)en_US
dc.subjectLiver Failureen_US
dc.subjectMortalityen_US
dc.titleHemophagocytic Lymphohistiocytosis in the Medical ICU: A Single-Institution Cohort Study on Acute Liver Failure and Mortalityen_US
dc.typeArticleen_US
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