Fulminant Gastrointestinal Bleeding caused by EBV-triggered Hemophagocytic Lymphohistiocytosis: Report of a Case

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare and often fatal hyperinflammatory syndrome characterized by fever, cytopenia, dramatically increased ferritin and hepatosplenomegaly. Here, we describe a previously healthy 39 year old pregnant woman in 30th week of her pregnancy with diarrhoea, intermittent gastrointestinal bleeding and fever of unknown focus. After cesarean section of twins in the 31st week she deteriorated with fulminant upper and lower gastrointestinal bleeding and disseminated intravascular coagulation. Gastro-, ileocolonoscopy and capsule endoscopy identified multiple bleeding punched ulcerations in the stomach, the entire small bowel and in parts of the colon. Emergency surgery with intraoperative endoscopy for uncontrolled hemorrhagic shock resulted in the resection of actively bleeding ulcers in the jejunum which temporally stabilized the critically ill patient. Jejunal histology and in situ hybridisation showed extensive ulcerations, focal lymphohistiocytic infiltration and EBV-positive immunoblasts. The diagnosis fulminant EBV-related HLH was confirmed based on the HLH-2004 diagnostic criteria and through detection of a reactivated EBV infection (up to 3 × 10(7) DNA copies/mL serum). Despite immunosuppressive therapy with steroids, cyclosporine A and etoposide in combination with Rituximab, the patient died from this sepsis-like, hyper-inflammatory syndrome in multiorgan failure with uncontrolled bleeding.