What we should know about hemophagocytic syndrom in the intensive care unit

Abstract

Hemophagocytic syndrome (HS) is a rare lifethreatening immunological disorder. Unregulated cytotoxic T and NK lymphocytic response induce a hyperinflammatory state. HS is characterized by fever, hepatomegaly or splenomegaly, cytopenias and may be responsible for multiple organ failure and shock. Hyperferritinemia, hypertriglyceridemia, and hypofibrinemia are commonly seen. Intensive care physicians should be aware of HS to promptly offer an adequate treatment. HS diagnostic evaluation should be aggressive as the treatment of the underlying cause is the key to prevent morbidity and mortality. In this review, we will discuss HS clinical signs, diagnostic criteria, etiologic diagnostic, process and treatments in adults, excluding genetic forms.