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Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by dysregulated activation and proliferation of lymphocytes with massive cytokine release, resulting in a sepsis-like syndrome with multiorgan failure. Primary HLH is a rare inherited disease in childhood, whereas secondary HLH can be induced by infections or malignancies. Diagnosis of secondary HLH is particularly challenging, as the syndrome can be induced by severe infections and shares symptoms with severe sepsis. Further, few data describe the prevalence of HLH in the ICU setting. To identify diagnostic and prognostic factors in secondary HLH, researchers retrospectively assessed data on 11,004 adult patients admitted to one ICU between January 2000 and August 2012.
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