{
  "abstract": "Background: 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.\n\nMethods: We retrospectively reviewed children with biopsy-confirmed HNL who fulfilled at least five of eight HLH-2004 criteria at Guangzhou Women and Children’s Medical Center from January 2020 to December 2025. We summarized patient-level clinical, laboratory, immunological, pathological, treatment, acute-outcome, and follow-up data.\n\nResults: Seven children were included (four boys; median age, 7 years; range, 1–13 years). Three patients met seven HLH-2004 criteria, two met six, and two met five. Fever, ≥2-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%–9.83%). 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, 7–73 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.\n\nConclusion: 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.",
  "abstract_shown": true,
  "affiliations": [
    "Department of Nutrition, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health , , ,",
    "Department of Emergency, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health , , ,",
    "Department of Allergy, Immunology and Rheumatology, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health , ,"
  ],
  "article_type": "research article",
  "arxiv": null,
  "author_total": 7,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Wen-ling Guo"
    },
    {
      "affs": [
        1
      ],
      "name": "Dan-ping Zhu"
    },
    {
      "affs": [
        1
      ],
      "name": "Li Huang"
    },
    {
      "affs": [
        1
      ],
      "name": "Qiu-yan Peng"
    },
    {
      "affs": [
        1
      ],
      "name": "Pei-qing Li"
    },
    {
      "affs": [
        2
      ],
      "name": "Song Zhang"
    },
    {
      "affs": [
        1
      ],
      "name": "De-feng Liang"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/77b4evd7ac5gv.bib",
  "cited_by": 0,
  "doi": "10.3389/fimmu.2026.1935403",
  "fulltext_url": "https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13661800/?tool=EBI",
  "id": "77b4evd7ac5gv",
  "issns": [
    "1664-3224"
  ],
  "landing_url": "https://europepmc.org/article/PMC/PMC13661800",
  "language": "eng",
  "licence": "CC-BY",
  "licence_label": "CC BY",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "openalex",
      "2720|Autoimmune and Inflammatory Disorders Research"
    ]
  ],
  "openalex": "W7214896401",
  "pdf_url": "https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13661800/pdf/?tool=EBI",
  "pmcid": "PMC13661800",
  "pmid": null,
  "published": "2026-09-30",
  "publisher": "Frontiers Media SA",
  "ris": "https://research.draconitic.com/paper/77b4evd7ac5gv.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T12:17:20Z",
      "native_id": "PMC:PMC13661800",
      "source": "europepmc",
      "url": "https://europepmc.org/article/PMC/PMC13661800"
    },
    {
      "first_seen": "2026-10-30T12:19:15Z",
      "native_id": "10.3389/fimmu.2026.1935403",
      "source": "crossref",
      "url": "https://www.frontiersin.org/articles/10.3389/fimmu.2026.1935403/full"
    }
  ],
  "subjects": [
    {
      "field": "biology",
      "origin": "issn:1664-3224",
      "subfield": "immunology"
    }
  ],
  "title": "Kikuchi-Fujimoto disease with hemophagocytic lymphohistiocytosis in children: a seven-patient case series",
  "title_text": "Kikuchi-Fujimoto disease with hemophagocytic lymphohistiocytosis in children: a seven-patient case series",
  "type": "peer-reviewed",
  "updated_at": "2026-10-30T12:19:15Z",
  "url": "https://research.draconitic.com/paper/77b4evd7ac5gv",
  "venue": "Frontiers in immunology",
  "versions": []
}