Intensive Care management of Hemophagocytic Lymphohistiocytosis: a case series

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BACKGROUND: Hemophagocytic Lymphohistiocytosis (HLH) is a syndrome characterized by severe hyperinflammation and immune dysfunction with concomitant immune system activation. HLH often requires supportive care management in Intensive Care Unit (ICU) with high mortality rates (36–40% of pediatric cases and 41–68% of adult cases). HLH diagnosis in ICU may be challenging due to lack of specific laboratory, radiologic and histopathologic findings, and above all the overlap of its clinical features with Multi-Organ Dysfunction syndrome (MOD). Here we present clinical characteristics, possible triggers and outcomes of a cohort of six patients admitted to our ICU for HLH or HLH-induced MOD. METHODS: Here we present clinical characteristics, possible triggers and outcomes retrospectively collected of a cohort of patients admitted to our ICU for HLH or HLH-induced MOD. The study has been approved by the local ethics committee as part of the PROSAFE data collection project. RESULTS: Six patients were included. All but one patient had a HScore with high probability of HLH, even when not all HScore items were tested. We found infection as a potential trigger in all patients. All patients died before hospital discharge. CONCLUSION: HLH is a condition that can overlap clinical features of septic shock. Although rare it should be considered in differential diagnosis of distributive shock without established etiology.