Clinicopathologic Heterogeneity and Prognostic Determinants in Pediatric Chronic Active Epstein-Barr Virus Infection

Area of Science:

• Hematology
• Oncology
• Virology

Background:

• Chronic active Epstein-Barr virus (CAEBV) infection is a rare, life-threatening lymphoproliferative disorder.
• It is driven by the Epstein-Barr virus and affects T/natural killer (NK) cells.
• CAEBV presents with diverse clinical and pathological features and lacks clear prognostic markers.

Purpose of the Study:

• To characterize the histopathologic spectrum of pediatric CAEBV.
• To evaluate the correlation between histopathologic features and clinical outcomes.

Main Methods:

• Retrospective analysis of 101 pediatric CAEBV patients.
• Assessment of clinical features, histopathologic grading (Grade-1, -2, -3), and immunophenotype.
• Evaluation of treatment responses and survival rates.

Main Results:

• Histopathologic grading revealed proliferative (52.5%), borderline (32.7%), and neoplastic (14.9%) lesions.
• Higher grades showed increased mixed T/NK-cell immunophenotypes and spatial heterogeneity.
• Pathologic grade significantly predicted treatment response and overall survival, while immunophenotype did not.

Conclusions:

• Histopathologic grading is a valuable prognostic tool for pediatric CAEBV.
• Multisite biopsies are essential for accurate risk stratification.
• Findings guide therapeutic strategies and improve patient outcomes.