Kikuchi-Fujimoto disease with hemophagocytic lymphohistiocytosis in children: a seven-patient case series
- 1Department of Nutrition, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health , , ,
- 2Department of Emergency, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health , , ,
- 3Department of Allergy, Immunology and Rheumatology, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health , ,
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.
Methods: 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.
Results: 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.
Conclusion: 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.
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BibTeX
@article{guo2026kikuchifujimoto,
title = {{Kikuchi-Fujimoto disease with hemophagocytic lymphohistiocytosis in children: a seven-patient case series}},
author = {Wen-ling Guo and Dan-ping Zhu and Li Huang and Qiu-yan Peng and Pei-qing Li and Song Zhang and De-feng Liang},
journal = {Frontiers in immunology},
year = {2026},
month = sep,
doi = {10.3389/fimmu.2026.1935403},
url = {https://europepmc.org/article/PMC/PMC13661800},
note = {CC BY},
}
RIS
TY - JOUR
TI - Kikuchi-Fujimoto disease with hemophagocytic lymphohistiocytosis in children: a seven-patient case series
AU - Wen-ling Guo
AU - Dan-ping Zhu
AU - Li Huang
AU - Qiu-yan Peng
AU - Pei-qing Li
AU - Song Zhang
AU - De-feng Liang
PY - 2026
DA - 2026/09/30
JO - Frontiers in immunology
DO - 10.3389/fimmu.2026.1935403
UR - https://europepmc.org/article/PMC/PMC13661800
AB - 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. Methods: 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. Results: 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. Conclusion: 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.
SN - 1664-3224
ER -