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
