Abstract
RATIONALE: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease characterized by an excessive systemic inflammatory response. HLH is classified as primary or secondary, where the latter may occur in association with many infections. However, no case of HLH has been previously associated with group b streptococcus (GBS) sepsis. PATIENT CONCERNS: We present a fatal case of HLH in a 5-year-old girl with GBS sepsis. DIAGNOSIS: The present patient met 5 of the HLH criteria: fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, and hyperferritinemia. GBS was identified in 2 sets of peripheral blood bacterial cultures. INTERVENTIONS: Empirical antibiotics, inotropes, and immunoglobulins were administered. OUTCOMES: The clinical course of the patient was fulminant and the patient died of septic shock 10 hours after admission to the hospital. LESSONS: We suggest GBS infection can cause HLH and early awareness of HLH associated with GBS infection and proper effective treatment are necessary to reduce mortality.