J Pediatr Intensive Care 2014; 03(04): 243-254
DOI: 10.3233/PIC-14106
Review Article
Georg Thieme Verlag KG Stuttgart – New York

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

Demet Demirkol
a   Department of Pediatrics, Pediatric Intensive Care Unit, Koç University School of Medicine, Istanbul, Turkey
Joseph Carcillo
b   Department of Critical Care Medicine, Supported by RO1 GM108618 from National Institutes of Health, University of Pittsburgh, Pittsburgh, PA, USA
› Author Affiliations

Subject Editor:
Further Information

Publication History

25 September 2014

24 October 2014

Publication Date:
28 July 2015 (online)


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.