Frontiers in Pediatrics (Jun 2021)
Clinical Analysis of Pediatric Systemic Juvenile Xanthogranulomas: A Retrospective Single-Center Study
- Hongyun Lian,
- Hongyun Lian,
- Hongyun Lian,
- Hongyun Lian,
- Ang Wei,
- Ang Wei,
- Ang Wei,
- Ang Wei,
- Lejian He,
- Ying Yang,
- Ying Yang,
- Ying Yang,
- Ying Yang,
- Honghao Ma,
- Honghao Ma,
- Honghao Ma,
- Honghao Ma,
- Liping Zhang,
- Liping Zhang,
- Liping Zhang,
- Liping Zhang,
- Yitong Guan,
- Yitong Guan,
- Yitong Guan,
- Yitong Guan,
- Qing Zhang,
- Qing Zhang,
- Qing Zhang,
- Qing Zhang,
- Dong Wang,
- Dong Wang,
- Dong Wang,
- Dong Wang,
- Zhigang Li,
- Zhigang Li,
- Zhigang Li,
- Zhigang Li,
- Rui Zhang,
- Rui Zhang,
- Rui Zhang,
- Rui Zhang,
- Tianyou Wang,
- Tianyou Wang,
- Tianyou Wang,
- Tianyou Wang
Affiliations
- Hongyun Lian
- Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing, China
- Hongyun Lian
- National Key Discipline of Pediatrics, Capital Medical University, Beijing, China
- Hongyun Lian
- Key Laboratory of Major Disease in Children, Ministry of Education, Beijing, China
- Hongyun Lian
- Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
- Ang Wei
- Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing, China
- Ang Wei
- National Key Discipline of Pediatrics, Capital Medical University, Beijing, China
- Ang Wei
- Key Laboratory of Major Disease in Children, Ministry of Education, Beijing, China
- Ang Wei
- Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
- Lejian He
- Department of Pathology, Beijing Children's Hospital Affiliated With Capital Medical University, National Center for Children's Health, Beijing, China
- Ying Yang
- Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing, China
- Ying Yang
- National Key Discipline of Pediatrics, Capital Medical University, Beijing, China
- Ying Yang
- Key Laboratory of Major Disease in Children, Ministry of Education, Beijing, China
- Ying Yang
- Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
- Honghao Ma
- Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing, China
- Honghao Ma
- National Key Discipline of Pediatrics, Capital Medical University, Beijing, China
- Honghao Ma
- Key Laboratory of Major Disease in Children, Ministry of Education, Beijing, China
- Honghao Ma
- Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
- Liping Zhang
- Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing, China
- Liping Zhang
- National Key Discipline of Pediatrics, Capital Medical University, Beijing, China
- Liping Zhang
- Key Laboratory of Major Disease in Children, Ministry of Education, Beijing, China
- Liping Zhang
- Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
- Yitong Guan
- Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing, China
- Yitong Guan
- National Key Discipline of Pediatrics, Capital Medical University, Beijing, China
- Yitong Guan
- Key Laboratory of Major Disease in Children, Ministry of Education, Beijing, China
- Yitong Guan
- Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
- Qing Zhang
- National Key Discipline of Pediatrics, Capital Medical University, Beijing, China
- Qing Zhang
- Key Laboratory of Major Disease in Children, Ministry of Education, Beijing, China
- Qing Zhang
- Hematologic Disease Laboratory, Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing, China
- Qing Zhang
- Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
- Dong Wang
- Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing, China
- Dong Wang
- National Key Discipline of Pediatrics, Capital Medical University, Beijing, China
- Dong Wang
- Key Laboratory of Major Disease in Children, Ministry of Education, Beijing, China
- Dong Wang
- Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
- Zhigang Li
- National Key Discipline of Pediatrics, Capital Medical University, Beijing, China
- Zhigang Li
- Key Laboratory of Major Disease in Children, Ministry of Education, Beijing, China
- Zhigang Li
- Hematologic Disease Laboratory, Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing, China
- Zhigang Li
- Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
- Rui Zhang
- Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing, China
- Rui Zhang
- National Key Discipline of Pediatrics, Capital Medical University, Beijing, China
- Rui Zhang
- Key Laboratory of Major Disease in Children, Ministry of Education, Beijing, China
- Rui Zhang
- Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
- Tianyou Wang
- Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing, China
- Tianyou Wang
- National Key Discipline of Pediatrics, Capital Medical University, Beijing, China
- Tianyou Wang
- Key Laboratory of Major Disease in Children, Ministry of Education, Beijing, China
- Tianyou Wang
- Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
- DOI
- https://doi.org/10.3389/fped.2021.672547
- Journal volume & issue
-
Vol. 9
Abstract
Objective: To investigate the clinical characteristics, treatment, and prognosis of children with systemic juvenile xanthogranuloma (JXG).Methods: Clinical data of children with JXG who were hospitalized in Beijing Children's Hospital, Capital Medical University, from January 2012 to December 2019 were retrospectively analyzed, including clinical manifestations, laboratory determinations, treatment, and prognosis of the children. Patients were treated with vindesine + prednisone as the first-line treatment and cytarabine + vindesine + dexamethasone ± cladribine as the second-line treatment.Results: Ten patients, including 8 males and 2 females, with a median of onset age of 1.95 (0.80–7.30) years, exhibited multi-system dysfunction. The median age of diagnosis was 2.45 (1.30–12.10) years. The most common location of extracutaneous lesions was the central nervous system (6 cases), followed by the lung (5 cases) and bone (4 cases). Nine patients underwent first-line chemotherapy, and 6 patients underwent second-line chemotherapy, including 5 patients with poorly controlled disease after first-line treatment. The median observation time was 29 (3–115) months. Nine patients survived, whereas one patient died of respiratory failure caused by pulmonary infection. At the end of follow-up, 7 patients were in active disease (AD)/regression state (AD-better), and 2 patients were in an AD/stable state (AD-stable). Three patients had permanent sequelae, mainly central diabetes insipidus. The rates of response to the first-line treatment and the second-line treatment were 40.0 and 66.7% respectively.Conclusion: The chemotherapy protocol for Langerhans cell histiocytosis (LCH) may be effective for patients with systemic JXG. Central nervous system involvement may not impact overall survival, but serious permanent sequelae may occur.
Keywords
