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

DOI Web Site PubMed
  • SAKO Masahiro
    The First Hospital of Welfare-Medical Association Department of Pediatric Hematology and Oncology, Osaka City General Hospital
  • ISHII Takefumi
    Ishii Children's Clinic Department of Pediatric Hematology and Oncology, Osaka City General Hospital
  • OKADA Keiko
    Department of Pediatric Hematology and Oncology, Osaka City General Hospital
  • MOCHIZUKI Takahiro
    Kibono Mori Developmental Growth Clinic
  • HARA Junichi
    Department of Pediatric Hematology and Oncology, Osaka City General Hospital
  • KUDO Ko
    Department of Pediatrics, Hirosaki University Graduate School of Medicine
  • IMASHUKU Shinsaku
    Department of Laboratory Medicine, Uji-Tokushukai Medical Center

Bibliographic Information

Other Title
  • 治療後に髄膜に広範囲に広がった若年性黄色肉芽腫に非血縁臍帯血移植が著効した小児のランゲルハンス細胞組織球症
  • 症例報告 治療後に髄膜に広範囲に広がった若年性黄色肉芽腫に非血縁臍帯血移植が著効した小児のランゲルハンス細胞組織球症
  • ショウレイ ホウコク チリョウ ゴ ニ ズイマク ニ コウハンイ ニ ヒロガッタ ジャクネンセイ オウショク ニクゲシュ ニ ヒケツエン サイタイケツ イショク ガ チョコウシタ ショウニ ノ ランゲルハンス サイボウ ソシキキュウショウ

Search this article

Abstract

<p>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®) and dexamethasone palmitate (Limethasone®). The size of the intracranial JXG masses gradually decreased after u-CBT and disappeared after 4 years. Currently, the patient is doing well at the age of 25 years and is receiving androgen replacement therapy.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 61 (5), 468-473, 2020

    The Japanese Society of Hematology

Keywords

Details 詳細情報について

Report a problem

Back to top