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Hemophagocytic Lymphohistiocytosis in Critically Ill Patients

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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

  • 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. 2019; 2465-2477, 133(23)

https://www.ncbi.nlm.nih.gov/pubmed/30992265

  • Hemophagocytic Lymphohistiocytosis: Potentially Underdiagnosed in Intensive Care Units

Shock. 2018; 149-155, 50(2)      

https://www.ncbi.nlm.nih.gov/pubmed/30010630

Scientific Awards

  • 3rd price of SIK Innovation Award 2020

Working Group

PD Dr. med. Gunnar Lachmann

Prof. Dr. med. Claudia Spies

Cornelia Knaak

Prof. Dr. med. Didier Keh

Dr. rer. medic. Clarissa von Haefen

Dr. rer. medic. Nadine Paeschke

Dr. med. Gerald Vorderwülbecke

Patrick Heeren

Widuri Kho

Peter Nyvlt

Friederike Schuster