Abstract
Neonatal sepsis manifests as a dysregulated release of acute phase reactants due to suspected or proven infectious etiology leading to a systemic inflammatory state. This immune dysregulation can trigger a cytokine storm leading to macrophage activation syndrome (MAS). Macrophage activation syndrome has been postulated to manifest as sepsis with multi-organ dysfunction. Prompt suspicion of MAS is important as immunomodulatory therapy can reduce mortality and morbidity. We present 2 preterm neonates with early onset neonatal sepsis, where appropriate antibiotic therapy and supportive measures were ineffective. Macrophage activation syndrome was suspected in these 2 cases due to worsening thrombocytopenia and transaminitis. Additional investigations of ferritin, triglyceride, fibrinogen, and NT-Pro BNP fulfilled the recent classification criteria for MAS. The addition of Intravenous immunoglobulin (IVIG) and methylprednisolone led to improvement in clinical and laboratory outcomes in 1 neonate; however, other succumbed to massive pulmonary and intracranial bleeding. Early immunomodulatory therapy in neonatal sepsis-induced MAS can reduce mortality and morbidity.