Kikuchi-Fujimoto disease in the Eastern Mediterranean zone

Area of Science:

• Pathology
• Immunology
• Rare Diseases

Background:

• Kikuchi-Fujimoto disease (KFD) is a rare, benign, and self-limiting lymphohistiocytic disorder.
• KFD's epidemiology and prevalence in specific ethnic groups, particularly those of Arab descent in the East Mediterranean region, are not well-documented.

Purpose of the Study:

• To review institutional cases of Kikuchi-Fujimoto disease (KFD) in individuals of Arab ethnicity.
• To analyze and compare KFD reports from East Mediterranean countries with global data.
• To investigate potential factors influencing KFD prevalence, including genetic susceptibility.

Main Methods:

• Retrospective study utilizing histopathology database for KFD diagnosis.
• Comprehensive review of patient medical records, including demographics, clinical presentation, laboratory findings, comorbidities, and management.
• Literature review and comparative analysis of published KFD cases from East Mediterranean countries and other global regions.

Main Results:

• Eleven cases (0.6%) of KFD were identified from 1968 lymph node biopsies.
• The mean age of affected patients was 32 years, with females comprising 73% of cases.
• The disease was self-limiting in 45% of patients; 34% required corticosteroid therapy. One case was associated with lymphoma, leading to mortality.
• Jordanians and other Mediterranean populations of Arab background exhibited low KFD rates.

Conclusions:

• Kikuchi-Fujimoto disease (KFD) appears to have low prevalence in Jordanian and broader Arab populations within the East Mediterranean.
• Significantly higher KFD prevalence in East Asians may be linked to genetic susceptibility.
• Early and accurate diagnosis of KFD is crucial for managing patient morbidity, despite diagnostic challenges.