Pediatric intestinal Behçet disease complicated by myeloid malignancies

Kiichiro Kanamitsu, Akira Shimada, Ritsuo Nishiuchi, Tomonari Shigemura, Yozo Nakazawa, Kenichi Koike, Yuichi Kodama, Yuichi Shinkoda, Yoshifumi Kawano, Kozo Yasui, Koji Sasaki, Ryosuke Kajiwara, Hirokazu Tsukahara, Atsushi Manabe

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Behçet disease (BD) is rarely seen in children. Its clinical manifestations are believed to differ between pediatric and adult patients. The characteristics of BD complicated by myelodysplastic syndrome (MDS) are well established for adult patients; however, because only a few cases of pediatric-onset BD complicated by MDS have been reported, its clinical characteristics remain unknown. We here retrospectively review pediatric-onset BD complicated by myeloid malignancies in Japan, having identified five such patients. All patients were female and had gastrointestinal involvements, but lacked both major features of BD, i.e., uveitis and association with HLA-B51. All patients had advanced MDS or acute myeloid leukemia and received chemotherapy followed by hematopoietic stem cell transplantation. These five cases suggest that intestinal BD and myeloid malignancies have one or more pathophysiological mechanisms in common.

Original languageEnglish
Pages (from-to)377-382
Number of pages6
JournalInternational journal of hematology
Volume105
Issue number3
DOIs
Publication statusPublished - Mar 1 2017

Keywords

  • Gastrointestinal involvements
  • Myeloid malignancies
  • Pediatric-onset Behçet disease

ASJC Scopus subject areas

  • Hematology

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    Kanamitsu, K., Shimada, A., Nishiuchi, R., Shigemura, T., Nakazawa, Y., Koike, K., Kodama, Y., Shinkoda, Y., Kawano, Y., Yasui, K., Sasaki, K., Kajiwara, R., Tsukahara, H., & Manabe, A. (2017). Pediatric intestinal Behçet disease complicated by myeloid malignancies. International journal of hematology, 105(3), 377-382. https://doi.org/10.1007/s12185-016-2127-7