Management of the critically ill child with the sepsis/hemophagocytic lymphohistiocytosis/macrophage activation syndrome overlap syndrome

Abstract

Hemophagocytic lymphohistiocytosis (HLH) or macrophage activation syndrome (MAS) is a frequently fatal disease, which can result in end-organ damage and death. This condition shares features with sepsis and systemic inflammatory response syndrome. Making a diagnosis of HLH can be challenging since most of the clinical and laboratory features of HLH are quite nonspecific. Timely diagnosis is critical to start therapy before damage by hypercytokinemia becomes irreversible. The treatment for patients with suspected acquired HLH/sepsis/systemic inflammatory response syndrome/multi organ dysfunction syndrome/ MAS overlap syndrome should be guided primarily by the severity of signs and symptoms, age of the patient, and underlying conditions. It is critical that the risks of treatment or non-treatment be weighed according to the clinical presentation of each patient. In this article, the authors discuss the diagnostic similarities between sepsis/HLH/MAS and management of the critically ill child with sepsis/HLH/MAS overlap syndrome.

Keywords

Hemophagocytic lymphohistiocytosis - macrophage activation syndrome - sepsis - multiple organ dysfunction syndrome - hyperferritinemia - hypercytokinemia