Hemophagocytic lymphohistiocytosis associated with Epstein–Barr virus infection: case report and literature review

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is rare and life threatening syndrome. There are only a few reported cases of HLH with GI symptoms. We describe the case of an 18 months old boy who presented with a history of fever for 40 days, abdominal distention and hepatosplenomegaly. Abdominal x-ray showed a pneumoperitoneum. Urgent laparotomy was done which revealed an isolated cecal perforation. The histopathological findings in the subsequent resected bowel was HLH with evidence of positive EBV Barr infection.

Serious consequences of Epstein‐Barr virus infection: Hemophagocytic lymphohistocytosis

“…Hemophagocytic syndrome (HPS), also known as hemophagocytic lymphohistocytosis (HLH), is divided into primary and secondary. Primary HLH called family HLH (FLH) as well, which usually appears in childhood, is generally associated with genetic defects of T lymphocytes and natural killer (NK) cell 1 . Common causes of secondary HLH are infections, malignancies, autoimmune diseases, immunodeficiency states, etc Epstein‐Barr virus‐associated hemophagocytic lymphohistocytosis (EBV‐HLH) is a non‐neoplastic disease caused by immune response disorders secondary to Epstein‐Barr virus (EBV) infection 2 .…”

Epstein-Barr virus-induced infection-associated hemophagocytic lymphohistiocytosis with acute liver injury: A case report

“…In contrast, HPS caused by EBV infection tends to have progressive exacerbations, has multiple-organ involvement, and can lead to death. Liver injury has been reported as an independent risk factor for mortality[ 9 ].…”