[Successful unrelated cord blood transplantation for extensive meningeal juvenile xanthogranuloma developing after treatment of Langerhans cell histiocytosis in a child].


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
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

Pagination

468-473

Auteurs

Masahiro Sako (M)

The First Hospital of Welfare-Medical Association.
Department of Pediatric Hematology and Oncology, Osaka City General Hospital.

Takefumi Ishii (T)

Ishii Children's Clinic.
Department of Pediatric Hematology and Oncology, Osaka City General Hospital.

Keiko Okada (K)

Department of Pediatric Hematology and Oncology, Osaka City General Hospital.

Takahiro Mochizuki (T)

Kibono Mori Developmental Growth Clinic.

Junichi Hara (J)

Department of Pediatric Hematology and Oncology, Osaka City General Hospital.

Ko Kudo (K)

Department of Pediatrics, Hirosaki University Graduate School of Medicine.

Shinsaku Imashuku (S)

Department of Laboratory Medicine, Uji-Tokushukai Medical Center.

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Classifications MeSH