BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening condition that has a poor prognosis due to the ensuing cytokine storm leading to severe organ damage. Current treatment guidelines suggest using a combination of steroid- and etoposide-based chemotherapy.

CASE REPORT: The authors present a case of a 41-year-old African-American female who presented with symptoms of foodborne illness and who developed multi-organ dysfunction. HLH was suspected because of poor response to broad-spectrum antibiotics with a constellation of findings, including cytopenia, hypofibrinogenemia, hypertriglyceridemia, and hyperferritinemia. Clinical improvement was noted after administration of intravenous immunoglobulin and dexamethasone while waiting for the soluble interleukin-2 receptor levels; therefore, chemotherapy was not administered. 

CONCLUSIONS: Despite the variable and poor prognosis of HLH, early treatment with steroids and immunosuppressive therapy is crucial to improving the survival rate. The inclusion of immunoglobulin therapy should be considered a treatment option for HLH.

Keywords: Immunoglobulins, Intravenous, Lymphohistiocytosis, Hemophagocytic, Streptococcus pneumoniae