{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T16:52:49Z","timestamp":1791305569925,"version":"4.3.1"},"reference-count":20,"publisher":"BMJ","issue":"10","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2026,10,1]]},"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:p>This report describes a gentleman, in his 40s, presenting with 3 weeks of fever, confusion, widespread erythematous crusted rash, weight loss and generalised lymphadenopathy. Secondary haemophagocytic lymphohistiocytosis (HLH) was diagnosed based on established criteria.<\/jats:p>\n                  <jats:p>Treatment with anakinra and dexamethasone (after lymph node biopsy) was initiated promptly. CT demonstrated extensive lymphadenopathy, raising concern of an underlying lymphoma. Autoimmune serology was positive but not diagnostic for a specific rheumatological disorder. Ultrasound-guided core needle lymph node biopsy ultimately confirmed Kikuchi-Fujimoto disease (KFD) as the underlying trigger of HLH. The patient demonstrated marked clinical improvement following immunosuppressive therapy.<\/jats:p>\n                  <jats:p>This case illustrates a rare association between KFD, a typically benign and self-limiting condition and secondary HLH. KFD should be considered in patients with fever of unknown origin and lymphadenopathy. This report highlights the diagnostic challenge posed by overlapping clinical and radiological features and emphasises that early recognition and treatment can significantly improve clinical outcomes.<\/jats:p>","DOI":"10.1136\/bcr-2026-275078","type":"journal-article","created":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T16:05:23Z","timestamp":1791302723000},"page":"e275078","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Haemophagocytic lymphohistiocytosis triggered by Kikuchi-Fujimoto disease: a diagnostic challenge"],"prefix":"10.1136","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-0159-2058","authenticated-orcid":false,"given":"Tanya","family":"Manish Arickatt","sequence":"first","affiliation":[{"name":"Haematology, Royal Free London NHS Foundation Trust, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-3519-5869","authenticated-orcid":false,"given":"Fatima","family":"Memon","sequence":"additional","affiliation":[{"name":"Haematology, Royal Free London NHS Foundation Trust, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-8560-1169","authenticated-orcid":false,"given":"Anagha","family":"Harikumar","sequence":"additional","affiliation":[{"name":"Haematology, Royal Free London NHS Foundation Trust, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-8520-6295","authenticated-orcid":false,"given":"Claire","family":"Hemmaway","sequence":"additional","affiliation":[{"name":"Haematology, Royal Free London NHS Foundation Trust, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6460-774X","authenticated-orcid":false,"given":"Khushnuma","family":"Mullanfiroze","sequence":"additional","affiliation":[{"name":"Haematology, Royal Free London NHS Foundation Trust, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2026,10,6]]},"reference":[{"key":"2026100609051732000_19.10.e275078.1","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1146\/annurev-pathol-020117-043625","article-title":"Hemophagocytic Lymphohistiocytosis","volume":"13","author":"Al-Samkari","year":"2018","journal-title":"Annu Rev Pathol Mech Dis"},{"key":"2026100609051732000_19.10.e275078.2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-03-278127"},{"key":"2026100609051732000_19.10.e275078.3","doi-asserted-by":"crossref","DOI":"10.1186\/1750-1172-1-18","article-title":"Kikuchi-Fujimoto disease","volume":"1","author":"Bosch","year":"2006","journal-title":"Orphanet J Rare Dis"},{"key":"2026100609051732000_19.10.e275078.4","doi-asserted-by":"crossref","first-page":"2274","DOI":"10.1182\/blood.2024026243","article-title":"HLH: diagnostics revisited and improved","volume":"144","author":"La Ros\u00e9e","year":"2024","journal-title":"Blood"},{"key":"2026100609051732000_19.10.e275078.5","doi-asserted-by":"crossref","first-page":"1400","DOI":"10.1002\/art.40930","article-title":"2019 European League Against Rheumatism\/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus","volume":"71","author":"Aringer","year":"2019","journal-title":"Arthritis Rheumatol"},{"key":"2026100609051732000_19.10.e275078.6","unstructured":"University College London Hospitals . Management of haemophagocytic lymphohistiocytosis (HLH) in adult and adolescent patients. 2024. Available: https:\/\/www.uclh.nhs.uk\/application\/files\/8117\/4955\/1864\/HLH_Guideline_April_2024.pdf"},{"key":"2026100609051732000_19.10.e275078.7","unstructured":"Getting It Right First Time (GIRFT), British Society for Rheumatology (BSR) . Haemophagocytic lymphohistiocytosis (HLH): guidance on the diagnosis, treatment, management and governance. NHS England; 2026. Available: https:\/\/gettingitrightfirsttime.co.uk\/wp-content\/uploads\/2026\/04\/HLH-Guide-UPDATED-April-2026.pdf"},{"key":"2026100609051732000_19.10.e275078.8","doi-asserted-by":"crossref","first-page":"e358","DOI":"10.1016\/S2665-9913(20)30096-5","article-title":"Silencing the cytokine storm: the use of intravenous anakinra in haemophagocytic lymphohistiocytosis or macrophage activation syndrome","volume":"2","author":"Mehta","year":"2020","journal-title":"Lancet Rheumatol"},{"key":"2026100609051732000_19.10.e275078.9","doi-asserted-by":"crossref","DOI":"10.3390\/diseases12110271","article-title":"Kikuchi-Fujimoto Disease: A Case Series and Review of the Literature","volume":"12","author":"Deb","year":"2024","journal-title":"Diseases"},{"key":"2026100609051732000_19.10.e275078.10","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-006-0230-5"},{"key":"2026100609051732000_19.10.e275078.11","doi-asserted-by":"publisher","DOI":"10.1016\/S1684-1182(10)60058-8"},{"key":"2026100609051732000_19.10.e275078.12","doi-asserted-by":"publisher","DOI":"10.1258\/0022215001906561"},{"key":"2026100609051732000_19.10.e275078.13","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjmed.2014.04.034"},{"key":"2026100609051732000_19.10.e275078.14","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-008-0902-4"},{"key":"2026100609051732000_19.10.e275078.15","doi-asserted-by":"crossref","DOI":"10.3389\/fped.2021.745506","article-title":"Clinical and Laboratory Characteristics of Kikuchi-Fujimoto Disease According to Age","volume":"9","author":"Kim","year":"2021","journal-title":"Front Pediatr"},{"key":"2026100609051732000_19.10.e275078.16","doi-asserted-by":"crossref","DOI":"10.3389\/fneur.2021.565387","article-title":"Case Report: Histiocytic Necrotizing Lymphadenitis (Kikuchi-Fujimoto Disease) Concurrent With Aseptic Meningitis","volume":"12","author":"Song","year":"2021","journal-title":"Front Neurol"},{"key":"2026100609051732000_19.10.e275078.17","first-page":"163","article-title":"Kikuchi-Fujimoto disease and systemic lupus erythematosus","volume":"9","author":"Baenas","year":"2016","journal-title":"Int Med Case Rep J"},{"key":"2026100609051732000_19.10.e275078.18","first-page":"383","article-title":"Kikuchi-Fujimoto Disease Complicated with Reactive Hemophagocytic Lymphohistiocytosis","volume":"70","author":"Nishiwaki","year":"2016","journal-title":"Acta Med Okayama"},{"key":"2026100609051732000_19.10.e275078.19","doi-asserted-by":"crossref","first-page":"72","DOI":"10.25259\/SRJHS_24_2023","article-title":"A rare presentation of kikuchi disease as hemophagocytic lymphohistiocytosis","volume":"3","author":"Kasarabada","year":"2024","journal-title":"SRJHS"},{"key":"2026100609051732000_19.10.e275078.20","doi-asserted-by":"crossref","DOI":"10.1186\/s13256-019-2100-1","article-title":"Kikuchi-Fujimoto disease associated with systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report","volume":"13","author":"Vithoosan","year":"2019","journal-title":"J Med Case Rep"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2026-275078","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T16:05:26Z","timestamp":1791302726000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2026-275078"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,10]]},"references-count":20,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2026,10,6]]},"published-print":{"date-parts":[[2026,10]]}},"alternative-id":["10.1136\/bcr-2026-275078"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2026-275078","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,10]]}}}