Kikuchi-Fujimoto disease
Primary Author(s)*
Sumire Kitahara, MD
WHO Classification of Disease
| Structure | Disease |
|---|---|
| Book | Haematolymphoid Tumours (5th ed.) |
| Category | T-cell and NK-cell lymphoid proliferations and lymphomas |
| Family | Tumour-like lesions with T-cell predominance |
| Type | N/A |
| Subtype(s) | Kikuchi-Fujimoto disease |
Related Terminology
| Acceptable | Histiocytic necrotizing lymphadenitis; Kikuchi disease; Kikuchi lymphadenitis |
| Not Recommended | N/A |
Gene Rearrangements
Kikuchi-Fujimoto disease does not have characteristic or recurrent genetic alterations.
| Driver Gene | Fusion(s) and Common Partner Genes | Molecular Pathogenesis | Typical Chromosomal Alteration(s) | Prevalence -Common >20%, Recurrent 5-20% or Rare <5% (Disease) | Diagnostic, Prognostic, and Therapeutic Significance - D, P, T | Established Clinical Significance Per Guidelines - Yes or No (Source) | Clinical Relevance Details/Other Notes |
|---|---|---|---|---|---|---|---|
| NA |
Individual Region Genomic Gain/Loss/LOH
Kikuchi-Fujimoto disease does not have characteristic or recurrent genetic alterations.
| Chr # | Gain, Loss, Amp, LOH | Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size] | Relevant Gene(s) | Diagnostic, Prognostic, and Therapeutic Significance - D, P, T | Established Clinical Significance Per Guidelines - Yes or No (Source) | Clinical Relevance Details/Other Notes |
|---|---|---|---|---|---|---|
| NA |
Characteristic Chromosomal or Other Global Mutational Patterns
Kikuchi-Fujimoto disease does not have characteristic or recurrent genetic alterations.
| Chromosomal Pattern | Molecular Pathogenesis | Prevalence -
Common >20%, Recurrent 5-20% or Rare <5% (Disease) |
Diagnostic, Prognostic, and Therapeutic Significance - D, P, T | Established Clinical Significance Per Guidelines - Yes or No (Source) | Clinical Relevance Details/Other Notes |
|---|---|---|---|---|---|
| NA |
Gene Mutations (SNV/INDEL)
Kikuchi-Fujimoto disease does not have characteristic or recurrent genetic alterations.
| Gene | Genetic Alteration | Tumor Suppressor Gene, Oncogene, Other | Prevalence -
Common >20%, Recurrent 5-20% or Rare <5% (Disease) |
Diagnostic, Prognostic, and Therapeutic Significance - D, P, T | Established Clinical Significance Per Guidelines - Yes or No (Source) | Clinical Relevance Details/Other Notes |
|---|---|---|---|---|---|---|
| NA |
Epigenomic Alterations
NA
Genes and Main Pathways Involved
Kikuchi-Fujimoto disease does not have characteristic or recurrent genetic alterations.
However, in a study using exome and transcriptome sequencing of lymph node tissue samples from KFD patients, fourteen single nucleotide polymorphisms were identified as candidate markers for the disease and found hundreds of genes with altered expression involving immune system, chromatin remodeling, transcription pathways.
| Gene; Genetic Alteration | Pathway | Pathophysiologic Outcome |
|---|---|---|
| NA |
Genetic Diagnostic Testing Methods
Genetic testing is non-contributory to rule-in a diagnosis of Kikuchi-Fujimoto disease. If the morphology and immunophenotype raise concern for lymphoma, particularly of T-lineage, genetic testing (e.g. T-cell receptor gene rearrangement, cytogenetic and/or NGS studies) may play a role.
Familial Forms
NA
Additional Information
Most published studies indicate a reactive, immune-mediated process rather than a neoplastic one. Some reports describe HLA class II associations (e.g., HLA-DPA1 and HLA-DPB1 polymorphisms)[1] in certain populations, suggesting a genetic susceptibility.[2]
Exomes and transcriptomes of lymph node tissue samples from KFD patients were analyzed by DNA sequencing. Fourteen single nucleotide polymorphisms (SNPs) were identified as candidate KFD markers and found hundreds of genes with altered expression (238 up, 1,519 down) involving immune system, chromatin remodeling, transcription pathways.
Links
NA
References
Notes
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*Citation of this Page: “Kikuchi-Fujimoto disease”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated 11/6/2025, https://ccga.io/index.php/HAEM5:Kikuchi-Fujimoto_disease.
- ↑ Tanaka, T.; et al. (1999-09). "DNA typing of HLA class II genes (HLA‐DR, ‐DQ and ‐DP) in Japanese patients with histiocytic necrotizing lymphadenitis (Kikuchi's disease)". Tissue Antigens. 54 (3): 246–253. doi:10.1034/j.1399-0039.1999.540305.x. ISSN 0001-2815. Check date values in:
|date=(help) - ↑ Isoda, Atsushi; et al. (2023-12). "Kikuchi-Fujimoto Disease in Human Leukocyte Antigen Partially Matched Siblings: A Case Study of Familial Susceptibility". Cureus. 15 (12): e51010. doi:10.7759/cureus.51010. ISSN 2168-8184. PMC 10803893 Check
|pmc=value (help). PMID 38264372 Check|pmid=value (help). Check date values in:|date=(help)