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A Malignant Mimicker: Features of Kikuchi-Fujimoto Disease in the Pediatric Population

Authors :
Karen M. Chisholm
Sandra D. Bohling
Karen D. Tsuchiya
Vera A. Paulson
Source :
Pediatric and Developmental Pathology. 25:538-547
Publication Year :
2022
Publisher :
SAGE Publications, 2022.

Abstract

Background Kikuchi-Fujimoto disease (KFD) is a rare, benign, and self-limited disease that presents with cervical lymphadenopathy and systemic symptoms. Histologic evaluation is often necessary to differentiate KFD from other entities. Methods Electronic medical records and diagnostic material were reviewed for 14 children diagnosed with KFD and 6 children diagnosed with infectious mononucleosis (IM) from 2013-2021. Four cases of KFD were further characterized using targeted DNA-based next-generation sequencing. Results Systemic symptoms were present in 86% (n = 12/14) of KFD patients, the most common being fever. Laboratory values worrisome for malignancy included cytopenia(s) (n = 9/12), elevated ESR and/or CRP (n = 9/12), elevated ferritin (n = 7/7), and elevated LDH (n = 7/10). Histologically, lymph nodes showed characteristic necrotic foci without neutrophils surrounded by MPO+ “crescentic” histiocytes. Immunoblasts and CD123+ plasmacytoid dendritic cells (pDCs) were also increased surrounding the necrosis. IM lymph nodes showed similar features when necrosis was present but increases in pDCs were patchy and rare neutrophils were seen in the necrotic foci. Molecular analysis of 4 KFD cases did not identify pathogenic variants. Conclusion While the signs/symptoms of KFD are worrisome, there are pathologic features that help differentiate it from potential mimics. We did not identify characteristic molecular features to aid in the work-up of these cases.

Details

ISSN :
16155742 and 10935266
Volume :
25
Database :
OpenAIRE
Journal :
Pediatric and Developmental Pathology
Accession number :
edsair.doi.dedup.....975fc409c791bea8a1482d8b3d5285ee
Full Text :
https://doi.org/10.1177/10935266221103882