Management of Hemophagocytic Lympho-Histiocytosis in Critically Ill Patients

Hemophagocytic syndrome remains a rare but life-threatening complication and is associated with intensive care unit (ICU) admission. The pathophysiology is based on a defect of cytotoxicity in T cells that results in a state of hyperinflammation in the presence of a trigger. As a consequence, patien...

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Bibliographic Details
Published in:Journal of Intensive Care Medicine 2020-02, Vol.35 (2), p.118-127
Main Authors: Lemiale, Virginie, Valade, Sandrine, Calvet, Laure, Mariotte, Eric
Format: Article
Language:English
Subjects:
Aged
Critical Care - methods
Disease Management
Female
Humans
Lymphohistiocytosis, Hemophagocytic - complications
Lymphohistiocytosis, Hemophagocytic - diagnosis
Lymphohistiocytosis, Hemophagocytic - therapy
Multiple Organ Failure - etiology
Multiple Organ Failure - therapy
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Summary:Hemophagocytic syndrome remains a rare but life-threatening complication and is associated with intensive care unit (ICU) admission. The pathophysiology is based on a defect of cytotoxicity in T cells that results in a state of hyperinflammation in the presence of a trigger. As a consequence, patients may develop multiorgan failure. The diagnosis of hemophagocytic syndrome (HS) remains difficult and relies on persistant high-grade fevers in the absence of infection and on constellation of laboratory parameters. However, prompt diagnosis and treatment (supportive care and specific treatment) are associated with improved outcome. Interaction with other specialists (hematologist, internist) may improve the diagnosis and treatment strategy. This article describes diagnostic tools, organ failures associated with HS, main etiologies, and management.
ISSN:0885-0666
1525-1489
DOI:10.1177/0885066618810403