| Literature DB >> 27848185 |
Kiichiro Kanamitsu1, Akira Shimada2, Ritsuo Nishiuchi3, Tomonari Shigemura4, Yozo Nakazawa4, Kenichi Koike4, Yuichi Kodama5, Yuichi Shinkoda5, Yoshifumi Kawano5, Kozo Yasui6, Koji Sasaki7, Ryosuke Kajiwara7, Hirokazu Tsukahara1, Atsushi Manabe8.
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.Entities:
Keywords: Gastrointestinal involvements; Myeloid malignancies; Pediatric-onset Behçet disease
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Year: 2016 PMID： 27848185 DOI： 10.1007/s12185-016-2127-7
Source DB: PubMed Journal: Int J Hematol ISSN： 0925-5710 Impact factor: 2.490