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Long-term complications in uniformly treated paediatric Langerhans histiocytosis patients disclosed by 12 years of follow-up of the JLSG-96/02 studies
- Source :
- British journal of haematologyReferences. 192(3)
- Publication Year :
- 2020
-
Abstract
- Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasia derived from immature myeloid dendritic cells with the mitogen-activated protein kinase (MAPK) pathway gene mutation. LCH is rarely fatal, but patients develop various permanent consequences (PCs). We report the frequencies of LCH-related PCs in paediatric patients (n = 317) treated by the JLSG-96/02 AraC-containing regimens. One-third of LCH patients had at least one PC at a median follow-up of 12 years. Central nervous system (CNS)-related PCs (neurological and endocrinological) accounted for 21·5%, non-CNS-related 16·7%. We require novel therapeutic measures to further reduce the frequency of LCH-related PCs.
- Subjects :
- Long term complications
MAPK/ERK pathway
Male
Pathology
medicine.medical_specialty
Myeloid
Adolescent
Central nervous system
Gene mutation
03 medical and health sciences
0302 clinical medicine
Langerhans cell histiocytosis
Central Nervous System Diseases
medicine
Humans
Protein kinase A
Child
business.industry
Cytarabine
Infant
Hematology
medicine.disease
Histiocytosis
Histiocytosis, Langerhans-Cell
medicine.anatomical_structure
030220 oncology & carcinogenesis
Child, Preschool
Female
business
Immunosuppressive Agents
030215 immunology
Follow-Up Studies
Subjects
Details
- ISSN :
- 13652141
- Volume :
- 192
- Issue :
- 3
- Database :
- OpenAIRE
- Journal :
- British journal of haematologyReferences
- Accession number :
- edsair.doi.dedup.....54095dff5d0c449dc4b651324dbd60bc