[Successful unrelated cord blood transplantation for extensive meningeal juvenile xanthogranuloma developing after treatment of Langerhans cell histiocytosis in a child].
Intracranial meningeal mass
Langerhans cell histiocytosis
Unrelated cord blood transplantation
Xanthogranuloma
Journal
[Rinsho ketsueki] The Japanese journal of clinical hematology
ISSN: 0485-1439
Titre abrégé: Rinsho Ketsueki
Pays: Japan
ID NLM: 2984782R
Informations de publication
Date de publication:
2020
2020
Historique:
entrez:
9
6
2020
pubmed:
9
6
2020
medline:
6
8
2020
Statut:
ppublish
Résumé
A 2-year and 4-month-old boy developed Langerhans cell histiocytosis (LCH) at the left parietal region of the skull. After treatment with chemotherapy, the patient achieved remission but experienced three relapses. After 3 years, he complained of headache, blurred vision, and lethargy. Brain magnetic resonance imaging revealed multiple dura-based meningeal masses. Biopsy was performed, and the patient was then diagnosed with juvenile xanthogranuloma (JXG). The analysis of both LCH/JXG tissues revealed BRAF V600E mutation. The JXG masses were not responsive to prednisolone, which was injected locally, radiotherapy (24 Gy), and chemotherapy (2-chlorodeoxy-adenosine). In addition, since the patient developed macrophage activation syndrome associated with systemic JXG progression, he received unrelated cord blood transplantation (u-CBT) at the age of 10 years and 11 months. Engraftment was performed at day 42, and significant GVHD was not observed. Four months after CBT, the patient was treated with infliximab (Remicade
Identifiants
pubmed: 32507810
doi: 10.11406/rinketsu.61.468
doi:
Types de publication
Case Reports
Journal Article
Langues
jpn
Sous-ensembles de citation
IM