Kikuchi-Fujimoto disease
Haematolymphoid Tumours (WHO Classification, 5th ed.)
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.
| 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. T-cell receptor gene rearrangement and/or NGS studies may play a role when morphology and immunophenotype raise concern for lymphoma.
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[1], suggesting a genetic susceptibility.
Links
NA
References
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Notes
*Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the Associate Editor or other CCGA representative. When pages have a major update, the new author will be acknowledged at the beginning of the page, and those who contributed previously will be acknowledged below as a prior author.
*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.