Infection-Associated Hemophagocytic Syndrome in Critically-Ill Patients: a Narrative Review

Abstract

The main clinical features of the hemophagocytic syndrome (HS) are febrile cytopenias with hepatosplenomegaly, usually in the setting of an underlying immune deficiency. This rare syndrome can be life-threatening, leading to organ dysfunctions which may require intensive care unit (ICU) management. HS in adults is mainly related to infections, hematological malignancies, and autoimmune diseases. Infections associated with HS are chiefly related to viruses, among which EBV, CMV, and other herpes viruses are the most common. HS has been also reported in patients with bacterial infections (mycobacterial infections), parasites (leishmaniasis, toxoplasmosis), and fungal infections (histoplasma). The management of infections associated with HS requires a triple approach: the treatment of the infection, the supportive treatments, and the specific treatments of the HS. These latter are mainly steroids, intravenous immunoglobulins, and etoposide. The decision to commence or not a treatment targeting the HS is guided by the patient’s condition (presence of organ dysfunctions). Another important aspect of the management is to investigate the patient for an underlying cause of immunosuppression. HIV infection, hematological malignancies (Hodgkin lymphoma, non-Hodgkin lymphomas, and Castleman’s disease), and autoimmune diseases (Lupus and Adult-onset Still’s disease) are the most causes of immunosuppression in patients with HS.