Pulmcrit (EMCrit) - June 6, 2016 by Josh Farkas
"Sepsis is a broadly defined syndrome, encompassing dozens of diseases. Could sepsis be split into smaller, more biologically homogeneous groups of patients, who might respond similarly to therapy? This could allow a more personalized approach to sepsis care.
Since the 1980s it has been recognized that some patients with sepsis also develop hemophagocytic lymphocytosis (Risdall 1984). For decades this was believed to be extremely rare. However, currently there is increasing recognition that this combination might represent a significant fraction of sepsis patients (Shakoory 2016, Beutel 2011)...
Summary
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome resulting from immunologic hyperactivation centering on macrophages. It may be caused by infection, cancer, or rheumatologic disease.
Clinically, HLH causes fever, shock, capillary leak, thrombocytopenia, delirium, disseminated intravascular coagulation, and multiorgan failure.
Patients with HLH due to infection have a combination of sepsis and HLH (sepsis-HLH overlap syndrome, SHLHOS). SHLHOS represents a subset of sepsis patients with intense immunologic hyperactivation and high mortality.
Ideal treatment for SHLHOS might be a combination of therapies for sepsis (e.g. antibiotics, supportive care) as well as immunosupressive therapies for HLH (e.g. steroid, possibly anakinra)."