{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T06:55:33Z","timestamp":1790751333360,"version":"4.1.0"},"reference-count":24,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:00:00Z","timestamp":1790726400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis (HNL), is typically self-limited but may be complicated by hemophagocytic lymphohistiocytosis (HLH) and may coexist with autoimmune disease, particularly systemic lupus erythematosus (SLE). Pediatric HNL-HLH is rare, and detailed data on pathology, HLH-2004 criteria, immune profiles, acute-care requirements, and subsequent autoimmune diagnoses remain limited.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We retrospectively reviewed children with biopsy-confirmed HNL who fulfilled at least five of eight HLH-2004 criteria at Guangzhou Women and Children\u2019s Medical Center from January 2020 to December 2025. We summarized patient-level clinical, laboratory, immunological, pathological, treatment, acute-outcome, and follow-up data.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Seven children were included (four boys; median age, 7 years; range, 1\u201313 years). Three patients met seven HLH-2004 criteria, two met six, and two met five. Fever, \u22652-lineage cytopenia, hyperferritinemia, and hypertriglyceridemia and\/or hypofibrinogenemia occurred in all patients. Antinuclear antibody was positive in three patients, and complement C3 and\/or C4 levels were low in four. Admission peripheral-blood natural killer (NK)-cell proportions were below the local reference range in five patients (range, 1.11%\u20139.83%). Four patients required pediatric intensive care, and three underwent plasma exchange. At discharge, complete remission was documented in six patients and partial remission in one; no in-hospital deaths occurred. Six patients had post-discharge follow-up (median, 23.5 months; range, 7\u201373 months), with no documented HLH recurrence. SLE was diagnosed in three patients during the index hospitalization, at 6, 11, and approximately 92 days after admission.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This seven-patient series documents intensive-care requirements in four children with HNL-HLH and clinical SLE diagnoses in three during the index hospitalization. These observations highlight the importance of pathological confirmation, assessment of hyperinflammatory severity, and rheumatologic follow-up when autoimmune features are present. Larger multicenter studies are needed to clarify the prognostic and therapeutic implications of immune-profile abnormalities.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fimmu.2026.1935403","type":"journal-article","created":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T06:00:58Z","timestamp":1790748058000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Kikuchi-Fujimoto disease with hemophagocytic lymphohistiocytosis in children: a seven-patient case series"],"prefix":"10.3389","volume":"17","author":[{"given":"Wen-ling","family":"Guo","sequence":"first","affiliation":[{"name":"Department of Nutrition, Guangzhou Women and Children\u2019s Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health","place":["Guangzhou, Guangdong, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dan-ping","family":"Zhu","sequence":"additional","affiliation":[{"name":"Department of Emergency, Guangzhou Women and Children\u2019s Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health","place":["Guangzhou, Guangdong, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Emergency, Guangzhou Women and Children\u2019s Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health","place":["Guangzhou, Guangdong, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qiu-yan","family":"Peng","sequence":"additional","affiliation":[{"name":"Department of Emergency, Guangzhou Women and Children\u2019s Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health","place":["Guangzhou, Guangdong, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pei-qing","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Emergency, Guangzhou Women and Children\u2019s Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health","place":["Guangzhou, Guangdong, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Song","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Allergy, Immunology and Rheumatology, Guangzhou Women and Children\u2019s Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health","place":["Guangzhou, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"De-feng","family":"Liang","sequence":"additional","affiliation":[{"name":"Department of Emergency, Guangzhou Women and Children\u2019s Medical Center, Guangzhou Medical 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