An unusual case of Kikuchi-Fujimoto disease presenting as haemophagocytic lymphohistiocytosis.

Abstract

British Journal of Hospital MedicineVol. 82, No. 9 Case ReportAn unusual case of Kikuchi–Fujimoto disease presenting as haemophagocytic lymphohistiocytosisKavina Shah, Raj Amarnani, Leah Owen, Jessica Manson, Alexis JonesKavina ShahDepartment of Rheumatology, University College London Hospital, London, UKSearch for more papers by this author, Raj AmarnaniDepartment of Rheumatology, University College London Hospital, London, UKSearch for more papers by this author, Leah OwenHospital for Tropical Diseases, University College London Hospital, London, UKSearch for more papers by this author, Jessica MansonDepartment of Rheumatology, University College London Hospital, London, UKSearch for more papers by this author, Alexis JonesCorrespondence to: Alexis Jones; E-mail Address: [email protected]Department of Rheumatology, University College London Hospital, London, UKSearch for more papers by this authorKavina Shah; Raj Amarnani; Leah Owen; Jessica Manson; Alexis JonesPublished Online:30 Aug 2021https://doi.org/10.12968/hmed.2021.0057AboutSectionsView articleView Full TextPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareShare onFacebookTwitterLinked InEmail View article References Bosch X, Guilabert A. Kikuchi-Fujimoto disease. Orphanet J Rare Dis. 2006;1(1):18. https://doi.org/10.1186/1750-1172-1-18 Crossref, Medline, Google ScholarFardet L, Galicier L, Lambotte O et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthr Rheumatol. 2014;66(9):2613–2620. https://doi.org/10.1002/art.38690 Crossref, Medline, Google ScholarFujimoto Y, Kozima Y, Yamaguchi K. Cervical subacute necrotizing lymphadenitis. A new clinicopathologic entity. Intern Med. 1972;20:920–927 Google ScholarHutchinson M, Tattersall RS, Manson JJ. Haemophagocytic lymphohisticytosis—an underrecognized hyperinflammatory syndrome. Rheumatology. 2019;58(Supplement_6):vi23–vi30. https://doi.org/10.1093/rheumatology/kez379 Crossref, Medline, Google ScholarKikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytes: a clinicopathological study. Acta Haematol Jpn. 1972;35:379–380 Google ScholarLim GY, Cho B, Chung NG. Hemophagocytic lymphohistiocytosis preceded by Kikuchi disease in children. Pediatr Radiol. 2008;38(7):756–761. https://doi.org/10.1007/s00247-008-0894-x Crossref, Medline, Google ScholarMahadeva U, Allport T, Bain B, Chan WK. Haemophagocytic syndrome and histiocytic necrotising lymphadenitis (Kikuchi's disease). J Clin Pathol. 2000;53(8):636–638. https://doi.org/10.1136/jcp.53.8.636 Crossref, Medline, Google ScholarNishiwaki M, Hagiya H, Kamiya T. Kikuchi-Fujimoto disease complicated with reactive hemophagocytic lymphohistiocytosis. Acta Medica Okayama. 2016;70(5):383–388. https://doi.org/10.18926/AMO/54597 Medline, Google ScholarToribio KA, Kamino H, Hu S, Pomeranz M, Pillinger MH. Co-occurrence of Kikuchi-Fujimoto's disease and Still's disease: case report and review of previously reported cases. Clin Rheumatol. 2015;34(12):2147–2153. https://doi.org/10.1007/s10067-014-2755-3 Crossref, Medline, Google ScholarVithoosan S, Karunarathna T, Shanjeeban P et al. Kikuchi-Fujimoto disease associated with systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report. J Med Case Rep. 2019; 13(1). https://doi.org/10.1186/s13256-019-2100-1 Crossref, Medline, Google ScholarWohlfarth P, Agis H, Gualdoni GA et al. Interleukin 1 receptor antagonist anakinra, intravenous immunoglobulin, and corticosteroids in the management of critically ill adult patients with hemophagocytic lymphohistiocytosis. J Intensive Care Med. 2019;34(9):723–731. https://doi.org/10.1177/0885066617711386 Crossref, Medline, Google Scholar FiguresReferencesRelatedDetails 2 September 2021Volume 82Issue 9ISSN (print): 1750-8460ISSN (online): 1759-7390 Metrics History Published online 30 August 2021 Published in print 2 September 2021 Information© MA Healthcare LimitedAcknowledgementsThe authors would like to thank Dr Sabine Pomplun, Consultant Histopathologist at University College London Hospital, for providing the histological diagnosis and histology slides to this case report as well as Dr Mike Brown, Consultant in Infectious Diseases and Tropical Medicine at University College London Hospital, for his contribution to the patient's clinical workup and management both in the inpatient and outpatient setting. They would also like to acknowledge Dr Anisha Bhagwanani, Senior Radiology Registrar at University College London Hospital, who provided the high-quality positron emission tomography–computed tomography images for this case report. They are very grateful to the patient for being so understanding during the extensive clinical work up required in his case and for providing consent to publish this report.PDF download