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Pathology of Kikuchi-Fujimoto disease - Top Ten Facts 

Dr Sampurna Roy MD   

 

                                                                                                                      

 

Histopathology:

Key microscopic features:

-Paracortical necrosis. Bright eosinophilic fibrinoid deposit.

- Abundant karyorrhectic debris. Karyorrhectic foci are formed by CD68+ histiocytes, plasmacytoid monocytes, immunoblasts, small and large lymphocytes, mainly CD8+ T lymphocytes.

- Histiocytes with crescent-shaped nuclei (crescentic nuclei),  histiocytes and macrophages containing phagocytozed debris from degenerated lymphocytes.

- Based on microscopic features, Kuo described three types of Kikuchi's disease: Proliferative, necrotizing, and xanthomatous.

 

(1) Kikuchi-Fujimoto disease (KFD), also called histiocytic necrotizing lymphadenitis, was initially reported in 1972 by Japanese pathologists Kikuchi and Fujimoto.  

They described the disease as "lymphadenitis with focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear debris."

(2) It is a rare, benign, and self-limited syndrome of unknown etiology characterized by tender localized lymphadenopathy, constitutional symptoms such as fever and night sweats. 

(3)  Kikuchi-Fujimoto disease occurs in patients during third and fourth decade of life and is usually more common in women than in men.

(4) The disease is more prevalent in Asian populations may be related to the presence of certain HLA alleles such as HLA class II alleles, HLA-DPA1 and HLA-DPB1, which are more prevalent in Asian patients.

(5) Although more prevalent in Asia,  some cases have been reported in other continents also.

(6)  The common laboratory abnormalities are leukopenia, usually neutropenia, anemia, thrombocytopenia , elevated C-reactive protein and erythrocyte sedimentation rate, impaired liver function and atypical lymphocytes on peripheral blood smear.

(7) Diagnosis is confirmed histopathologically.

In Kikuchi-Fujimoto disease,the most common histologic finding is lymph node showing geographic necrosis with foci of apoptotic cells with abundant karyorrhectic fragments surrounded by histiocytes. Characteristically, neutrophils and eosinophils are absent.

(8)  Etiology of Kikuchi-Fujimoto disease has remained unknown, although various infectious agents have been suspected like Yersinia enterocolitica, Brucella, Bartonella henselae, Entamoeba histolytica, Toxoplasma gondii. and viruses such as Epstein-Barr virus, herpes virus, cytomegalovirus, parvovirus, paramyxovirus, parainfluenza virus, Rubella, hepatitis-B, human immunodeficiency virus (HIV), human T-lymphotropic virus type-1 (HTLV-1), and the Dengue virus.

(9) Because of the clinical and pathological correlation between Kikuchi-Fujimoto disease and Systemic Lupus Erythematosus, some authors have postulated that KFD may be a clinical feature or an incomplete phase of lupus lymphadenitis.

(10)  Clinical and histological differential diagnoses of Kikuchi-Fujimoto disease include non-Hodgkin lymphomas and other lymphoid malignancies, lymphadenopathies associated with connective disorders such as SLE, rheumatoid arthritis, and Still’s disease and bacterial or viral infections such as cat scratch disease, infectious mononucleosis, herpes simplex, HIV, toxoplasmosis, tuberculosis, and atypical mycobacterial lymphadenitis.

 

Source

Kikuchi-Fujimoto Disease: Unusual Presentation of Rare Disease. Clinics and Practice. 2016;6(1):828.

The Enigmatic Kikuchi-Fujimoto Disease: A Case Report and Review. Case Reports in Hematology. 2014;2014:648136. 

Kikuchi–Fujimoto disease and systemic lupus erythematosus. International Medical Case Reports Journal. 2016;9:163-167

Kikuchi-Fujimoto Disease in Michigan: A Rare Case Report and Review of the Literature

 

 


 

 

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