Pediatric macrophage activation syndrome/sepsis-associated hemophagocytic lymphohistiocytosis: a diagnostic and therapeutic challenge / 中国小儿急救医学

ABSTRACT

It is an important turning point that early diagnosis and prompts initiation of adequate treatment improve the outcomes of primary or secondary hemophagocytic lymphohistiocytosis (HLH) which is a rare life-threatening syndrome.Even though the survival rate of the patients with HLH has been improved because of the immunochemotherapy or targeted immunotherapy based on new findings in the immunopathogenesis and the genetic defects,reducing the mortality will be main challenge for the pediatricians in pediatric intensive care unit.All of the clinical features are significant clues for the diagnosis,including continuing fever,specific liver dysfunction with coagulation abnormalities,pancytopenia,hypertriglyceridemia and hyperferritinemia.Defining patients' natural killer cells status during the different stages of sepsis and autoimmune diseases will be guiding innovative immunointerventions.