{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T09:10:12Z","timestamp":1756285812713,"version":"3.44.0"},"reference-count":10,"publisher":"BMJ","issue":"3","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2024,12,2]]},"published-print":{"date-parts":[[2025,3]]},"abstract":"<jats:p>Haemophagocytic lymphohistiocytosis (HLH) is a severe systemic inflammatory syndrome. Over activation of histiocytes and lymphocytes can lead to multiorgan failure and death. The three key features are fever, raised ferritin and falling counts, that is, cytopenia (3Fs). Treatment is immunosuppression while identifying the driver. Our young female patient presented with high fever and extensive lymphadenopathy, raising the suspicion of HLH driven by lymphoma. Her H-score was consistent with HLH. Immunosuppression was commenced with intravenous steroids, followed by anakinra due to inadequate steroid response. She required intensive care unit (ICU) admission with input from haematology and rheumatology. A lymph node biopsy revealed likely Kikuchi-Fujimoto disease, a rare benign cause of painful lymphadenopathy that is usually self-limiting but is also reported as a cause of HLH. She responded rapidly to anakinra and was discharged home 1\u2009week after leaving ICU. Regular clinic follow-up continues, immunosuppression has been weaned off and she remains in remission. Monitoring for later development of autoimmune disease continues. We discuss her management with reference to the recently published Getting it Right First Time guidelines on HLH.<\/jats:p>","DOI":"10.1136\/bcr-2024-263008","type":"journal-article","created":{"date-parts":[[2025,3,13]],"date-time":"2025-03-13T08:06:24Z","timestamp":1741853184000},"page":"e263008","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Emergency admission with unremitting fever and lymphadenopathy: Kikuchi-Fujimoto disease causing haemophagocytic lymphohistiocytosis"],"prefix":"10.1136","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-8965-0494","authenticated-orcid":false,"given":"Ayesha","family":"Mashadi","sequence":"first","affiliation":[{"name":"Haematology, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sarah","family":"Fordham","sequence":"additional","affiliation":[{"name":"Haematology, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-4863-7445","authenticated-orcid":false,"given":"Victoria Clare","family":"Willimott","sequence":"additional","affiliation":[{"name":"Norwich Medical School, University of East Anglia, Norwich, UK"},{"name":"Haematology, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2025,3,13]]},"reference":[{"doi-asserted-by":"crossref","unstructured":"Bosch X , Guilabert A . Kikuchi-Fujimoto disease. Orphanet J Rare Dis 2006;1. doi:10.1186\/1750-1172-1-18","key":"2025082701483660000_18.3.e263008.1","DOI":"10.1186\/1750-1172-1-18"},{"doi-asserted-by":"publisher","key":"2025082701483660000_18.3.e263008.2","DOI":"10.1182\/blood-2015-01-551622"},{"unstructured":"Guideline U . Diagnosis and management of haemophagocytic lymphohistiocytosis (hlh). Nhs.uk. n.d. Available: https:\/\/www.uclh.nhs.uk\/application\/files\/2017\/1558\/8681\/UCLH_HLH_guidelines.pdf","key":"2025082701483660000_18.3.e263008.3"},{"key":"2025082701483660000_18.3.e263008.4","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"},{"unstructured":"Lanyon P , Manson J , Tattersall R , et al . Guidance on the diagnosis, treatment, management and governance. GIRFT; 2004. Available: https:\/\/gettingitrightfirsttime.co.uk\/wp-content\/uploads\/2024\/07\/HLH-Guide-final-version-v1.1-July-2024.pdf","key":"2025082701483660000_18.3.e263008.5"},{"unstructured":"HScore for reactive hemophagocytic syndrome. MDCalc. n.d. Available: https:\/\/www.mdcalc.com\/calc\/10089\/hscore-reactive-hemophagocytic-syndrome","key":"2025082701483660000_18.3.e263008.6"},{"doi-asserted-by":"crossref","unstructured":"Chen Q , Zhang J , Huang H , et al . Histiocytic necrotizing lymphadenitis with hemophagocytic lymphohistiocytosis in adults: A single\u2010center analysis of 5 cases. Immun Inflamm Dis 2024;12. doi:10.1002\/iid3.1202","key":"2025082701483660000_18.3.e263008.7","DOI":"10.1002\/iid3.1202"},{"unstructured":"WHO Classification of Tumours Editorial Board . Hematolymphoid tumors. In: WHO Classification of Tumors Series. 5th edn. Lyon (France): International Agency for Research on Cancer, 2022. Available: https:\/\/tumourclassification.iarc.who.int","key":"2025082701483660000_18.3.e263008.8"},{"key":"2025082701483660000_18.3.e263008.9","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1002\/art.42750","article-title":"single\u2010cell Profiling of Bone Marrow B Cells and Early B Cell Developmental Disorders Associated With Systemic Lupus Erythematosus","volume":"76","author":"Dong","year":"2024","journal-title":"Arthritis & Rheumatology"},{"unstructured":"Clinical commissioning policy: anakinra for haemophagocytic lymphohistiocytosis (hlh) for adults and children in all ages [210701p] (1924). Commissioning position; 1924. Available: https:\/\/www.england.nhs.uk\/wp-content\/uploads\/2021\/10\/1924-Clinical-commissioning-policy-anakinra-for-haemophagocytic-lymphohistiocytosis-.pdf","key":"2025082701483660000_18.3.e263008.10"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2024-263008","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T08:49:05Z","timestamp":1756284545000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2024-263008"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,3]]},"references-count":10,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2025,3,13]]},"published-print":{"date-parts":[[2025,3]]}},"alternative-id":["10.1136\/bcr-2024-263008"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2024-263008","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2025,3]]}}}