Hemophagocytic Lymphohistiocytosis (HLH) is a rare, hyperinflammatory syndrome due to pathologic immune activation. HLH in the neonatal period is extremely rare. We present the case of a critically ill newborn full-term via urgent cesarean delivery for concerns for hydrops fetalis, ultimately found to have familial HLH. HLH carries a high fatality rate and poor prognosis. A high clinical suspicion of HLH and early initiation of treatment is crucial to survival. In cases where no amount of medical treatment is likely to be more than palliative, early discussions of goals of care is imperative to avoid unnecessary treatments.
Published Date: 2023-06-05; Received Date: 2023-05-04