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Hemophagocytic Lymphohistiocytosis in Critically Ill Patients
Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening hyperinflammatory syndrome with a high mortality rate of 60%, but is often not diagnosed due to the sepsis-like symptoms. It is assumed that HLH is a relevant but undetected cause of death in intensive care units. Early diagnosis and therapy are crucial for the outcome; without specific immunosuppressive treatment, HLH in most cases leads to death. While congenital HLH has been well studied in pediatric patients due to several genetic defects, there is little evidence of HLH in adults. Our working group systematically investigates HLH in critically ill patients. The aim is to develop new clinical concepts to diagnose HLH at an early stage and to reduce the fatal consequences.
Projects
HEMICU Study: The aim of this prospective observational study is to develop biomarkers that are highly sensitive and highly specific for HLH in adult critically ill patients.
ClinicalTrials.gov Identifier: NCT03510650
Study protocol: https://www.ncbi.nlm.nih.gov/pubmed/31666276
FERRITS Study: The aim of this exploratory registry study is to investigate laboratory, imaging, cytopathological and outcome parameters in the routine treatment of adult critically ill patients. The influence of ferritin levels on the outcome should be investigated with regard to HLH.
ClinicalTrials.gov Identifier: NCT02854943
Selected Publications
- Treatment and Mortality of Hemophagocytic Lymphohistiocytosis in Adult Critically Ill Patients: A Systematic Review With Pooled Analysis
Crit Care Med. 2020;10.1097/CCM.0000000000004581
https://www.ncbi.nlm.nih.gov/pubmed/32947471/ - Hemophagocytic lymphohistiocytosis in critically ill patients: diagnostic reliability of HLH-2004 criteria and HScore
Crit Care. 05/2020;24(1):244
https://www.ncbi.nlm.nih.gov/pubmed/32448380/ - Hyperferritinemia in Critically Ill Patients
Critical Care Medicine. 2019;10.1097/CCM.0000000000004131
https://www.ncbi.nlm.nih.gov/pubmed/31876528 - Hemophagocytic Lymphohistiocytosis in Critically Ill Patients
Shock. 2019;10.1097/SHK.0000000000001454
https://www.ncbi.nlm.nih.gov/pubmed/31626037 - Recommendations for management of hemophagocytic lymphohistiocytosis in adults
Blood. 06/2019, 2465-2477, 133(23)
https://www.ncbi.nlm.nih.gov/pubmed/30992265 - Hemophagocytic Lymphohistiocytosis: Potentially Underdiagnosed in Intensive Care Units
Shock, 08/2018, 149-155, 50(2)
https://www.ncbi.nlm.nih.gov/pubmed/30010630
