Abstract

Background: Hemophagocytic lymphohistiocytosis (HLH) is a result of dysregulated cellular response system. Primary HLH is an autosomal recessive disorder of childhood, with defects in cellular cytotoxicity. Secondary HLH is an acquired syndrome that presents in young adulthood secondary to a variety of inflammatory conditions: viral infections, rheumatologic conditions, or malignant processes. The inflammatory nature of certain conditions triggers a cytokine release in individuals who have abnormal T cell activation.
Case Report: A 30-year-old Hispanic male presented with worsening abdominal pain for 5 months and was found to have fever, pancytopenia, and hypotension. Serial CT scans of the abdomen/ pelvis showed splenomegaly but no abscesses, areas of infection, or masses. Infectious causes were considered but results of all cultures and tests were negative except for a high Epstein-Barr viral load. The patient deteriorated and required intubation on hospital day 28. Repeat bone marrow biopsy on day 32 suggested a diagnosis of hemophagocytic lymphohistiocytosis, although there was no evidence of hemophagocytosis within the bone marrow. The patient continued to deteriorate and was too unstable to receive treatment with chemotherapy. He died on hospital day 34.
Conclusions: This case highlights the importance of early consideration and treatment of secondary HLH in an individual presenting with progressive fever, hepatomegaly, and cytopenias.

Keywords: EBV, EBV associated with hemophagocytic lymphohistiocytosis , Hemophagocytic lymphohistiocytosis