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A Metadata Extraction Approach for Clinical Case Reports to Enable Advanced Understanding of Biomedical Concepts

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Systemic juvenile xanthogranuloma: A systematic review.

Tong Zou¹, Ang Wei¹, Honghao Ma¹

¹Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Disease in Children, Ministry of Education, Beijing children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.

Pediatric Blood & Cancer

|February 13, 2023

Summary

Systemic juvenile xanthogranuloma (SJXG) commonly affects the CNS, liver, and lungs, with younger onset linked to more organ involvement. Individualized therapy is crucial as no standard treatment exists for SJXG.

Keywords:

congenital outcome systemic juvenile xanthogranuloma treatment

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Area of Science:

Pediatric Oncology
Dermatology
Systemic Juvenile Xanthogranuloma

Background:

Systemic juvenile xanthogranuloma (SJXG) is a rare multisystemic non-Langerhans cell histiocytosis.
Understanding SJXG's epidemiology, clinical course, and outcomes is vital for effective management.

Approach:

A systematic review of literature published between 1981 and 2022 was conducted.
Data from 103 articles on 159 patients were analyzed for demographics, organ involvement, treatment, and outcomes.

Key Points:

Median onset age was 9 months, with a 61% male predominance.
The central nervous system (40.9%), liver (31.4%), and lungs (18.9%) were most frequently involved.
Spleen involvement significantly correlated with a higher risk of mortality (p=0.0003).

Conclusions:

SJXG presents with diverse extracutaneous site involvement.
No established therapy exists; individualized treatment approaches are recommended.
Permanent sequelae often involve central nervous system and ocular manifestations.