title: Pediatric intestinal Behçet disease complicated by myeloid malignancies.
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.
authors.affiliation: Department of Pediatric Hematology/Oncology, Okayama University Hospital, Okayama, 2-5-1, Shikatacho, Kitaku, Okayama, 700-8558, Japan.
authors.forename: Kiichiro
authors.initials: K
authors.lastname: Kanamitsu
authors.affiliation: Department of Pediatric Hematology/Oncology, Okayama University Hospital, Okayama, 2-5-1, Shikatacho, Kitaku, Okayama, 700-8558, Japan. pajj236e@okayama-u.ac.jp.
authors.forename: Akira
authors.identifier: http://orcid.org/0000-0001-5207-3779
authors.initials: A
authors.lastname: Shimada
authors.affiliation: Department of Pediatrics, Kochi Health Sciences Center, Kochi, Japan.
authors.forename: Ritsuo
authors.initials: R
authors.lastname: Nishiuchi
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: Tomonari
authors.initials: T
authors.lastname: Shigemura
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: Yozo
authors.initials: Y
authors.lastname: Nakazawa
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Koike
authors.affiliation: Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Kodama
authors.affiliation: Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Shinkoda
authors.affiliation: Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Kawano
authors.affiliation: Department of Pediatrics, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan.
authors.forename: Kozo
authors.initials: K
authors.lastname: Yasui
authors.affiliation: Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Sasaki
authors.affiliation: Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
authors.forename: Ryosuke
authors.initials: R
authors.lastname: Kajiwara
authors.affiliation: Department of Pediatric Hematology/Oncology, Okayama University Hospital, Okayama, 2-5-1, Shikatacho, Kitaku, Okayama, 700-8558, Japan.
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Tsukahara
authors.affiliation: Department of Pediatrics, St. Luke's International Hospital, Tokyo, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Manabe
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D001528:Behcet Syndrome / Q000150:complications* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007410:Intestinal Diseases / Q000150:complications* / Q000628:therapy; D007564:Japan; D015470:Leukemia, Myeloid, Acute / Q000150:complications* / Q000628:therapy; D009190:Myelodysplastic Syndromes / Q000150:complications* / Q000628:therapy; D012189:Retrospective Studies
chemical_list: D000970:Antineoplastic Agents
keywords: Gastrointestinal involvements; Myeloid malignancies; Pediatric-onset Behçet disease