Extranodal NK/T-cell lymphoma presenting as fatal gastrointestinal bleeding and haemophagocytic lymphohistiocytosis.

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome that should be considered in patients with persistent fevers. Here, we present a patient with a clinical diagnosis of Behçet's disease who presented acutely with rectal bleeding secondary to acute intraluminal ileal haemorrhage. During her admission, she developed HLH with fevers, hyperferritinaemia, cytopaenia and coagulopathy. Despite treatment with intravenous corticosteroids and anakinra, her condition deteriorated rapidly, and she died 16 days later. After her death, the diagnosis of extranodal NK/T-cell lymphoma was made based on bone marrow biopsy and bowel histopathology. This case highlights the importance of early consideration of HLH in patients with fevers, falling cell counts and hyperferritinaemia and identification of the underlying aetiology. Through this case report, we discuss the diagnostic challenges and importance of a multidisciplinary approach.