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. 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) in certain populations, suggesting a genetic susceptibility.

Links

NA

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.