TY - JOUR
T1 - Pediatric intestinal Behçet disease complicated by myeloid malignancies
AU - Kanamitsu, Kiichiro
AU - Shimada, Akira
AU - Nishiuchi, Ritsuo
AU - Shigemura, Tomonari
AU - Nakazawa, Yozo
AU - Koike, Kenichi
AU - Kodama, Yuichi
AU - Shinkoda, Yuichi
AU - Kawano, Yoshifumi
AU - Yasui, Kozo
AU - Sasaki, Koji
AU - Kajiwara, Ryosuke
AU - Tsukahara, Hirokazu
AU - Manabe, Atsushi
N1 - Funding Information:
This study was supported in part by a Grant-in-Aid for Cancer Research and a grant for Clinical Cancer Research and Research on Children and Families from the Ministry of Health, Labour and Welfare of Japan. The authors declare no conflict of interest associated with this manuscript.
Publisher Copyright:
© 2016, The Japanese Society of Hematology.
PY - 2017/3/1
Y1 - 2017/3/1
N2 - 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.
AB - 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.
KW - Gastrointestinal involvements
KW - Myeloid malignancies
KW - Pediatric-onset Behçet disease
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U2 - 10.1007/s12185-016-2127-7
DO - 10.1007/s12185-016-2127-7
M3 - Article
C2 - 27848185
AN - SCOPUS:84995476579
VL - 105
SP - 377
EP - 382
JO - International Journal of Hematology
JF - International Journal of Hematology
SN - 0925-5710
IS - 3
ER -