Affiliations: Department of Pediatrics, Pediatric Intensive Care Unit, Koç University School of Medicine, Istanbul, Turkey | Department of Critical Care Medicine, Supported by RO1 GM108618 from National Institutes of Health, University of Pittsburgh, Pittsburgh, PA, USA
Note:  Corresponding author: Demet Demirkol, MD, Koç University School of Medicine, Maltepe Mah. Davutpacşa Cad. No:4, Topkapi, İstanbul, Turkey. Tel.: +90 5326451474; Fax: +90 212 311 33 50; E-mails: [email protected]; [email protected]
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.