2018年12月13日
Cladribine treatment for Erdheim-Chester disease involving the central nervous system and concomitant polycythemia vera: A case report.
Journal of clinical and experimental hematopathology : JCEH
- 巻
- 58
- 号
- 4
- 開始ページ
- 161
- 終了ページ
- 165
- 記述言語
- 英語
- 掲載種別
- 研究論文(学術雑誌)
- DOI
- 10.3960/jslrt.18015
Erdheim-Chester disease (ECD), a rare form of non-Langerhans cell histiocytosis, is characterized by the infiltration of foamy CD68+ and CD1a- histiocytes into multiple organ systems. Central nervous system (CNS) involvement has recently been reported to be a poor prognostic factor when treating ECD with interferon alpha. We report the case of a 66-year-old Japanese patient with ECD involving the CNS who harbored the BRAF V600E mutation and also concomitantly developed polycythemia vera with the JAK2 V617F mutation. We confirmed 2-chlorodeoxyadenosine (cladribine) therapy to be effective for the patient in this case.
- リンク情報
- ID情報
-
- DOI : 10.3960/jslrt.18015
- ISSN : 1346-4280
- PubMed ID : 30305475
- PubMed Central 記事ID : PMC6407475