{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T02:08:59Z","timestamp":1777860539819,"version":"3.51.4"},"reference-count":9,"publisher":"BMJ","issue":"4","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2025,3,11]]},"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:p>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 Beh\u00e7et\u2019s 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.<\/jats:p>","DOI":"10.1136\/bcr-2024-263858","type":"journal-article","created":{"date-parts":[[2025,4,7]],"date-time":"2025-04-07T16:20:51Z","timestamp":1744042851000},"page":"e263858","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Extranodal NK\/T-cell lymphoma presenting as fatal gastrointestinal bleeding and haemophagocytic lymphohistiocytosis"],"prefix":"10.1136","volume":"18","author":[{"given":"Neha","family":"Maini","sequence":"first","affiliation":[{"name":"Rheumatology, Guy's and St Thomas\u2019 Hospitals NHS Trust, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5267-200X","authenticated-orcid":false,"given":"Kathryn","family":"Biddle","sequence":"additional","affiliation":[{"name":"Rheumatology, Guy's and St Thomas\u2019 Hospitals NHS Trust, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Green","sequence":"additional","affiliation":[{"name":"Cellular Pathology, Guy's and St Thomas\u2019 Hospitals NHS Trust, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michelle","family":"Fernando","sequence":"additional","affiliation":[{"name":"Rheumatology, Guy's and St Thomas\u2019 Hospitals NHS Trust, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanni","family":"Sanna","sequence":"additional","affiliation":[{"name":"Rheumatology, Guy's and St Thomas\u2019 Hospitals NHS Trust, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2025,4,7]]},"reference":[{"key":"2025082800463391000_18.4.e263858.1","doi-asserted-by":"crossref","first-page":"e51","DOI":"10.1016\/S2665-9913(23)00273-4","article-title":"Diagnosis and investigation of suspected haemophagocytic lymphohistiocytosis in adults: 2023 Hyperinflammation and HLH Across Speciality Collaboration (HiHASC) consensus guideline","volume":"6","author":"Cox","year":"2024","journal-title":"Lancet Rheumatol"},{"key":"2025082800463391000_18.4.e263858.2","first-page":"1414","article-title":"Haemophagocytic syndrome in rheumatic patients. 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