Pediatric Intestinal Behçet Disease Complicated by Myeloid Malignancies

Overview

Journal Int J Hematol

Specialty Hematology

Date 2016 Nov 17

PMID 27848185

Citations 4

Authors

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

Affiliations

Soon will be listed here.

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.

Citing Articles

Risk Factors of Reoperation in Patients with Intestinal Behçet's Disease Treated by Initial Bowel Resection.

Kim S, Park E, Bae H, Lee Y, Park M, Yang S J Clin Med. 2024; 13(22).

PMID: 39597915 PMC: 11594750. DOI: 10.3390/jcm13226771.

Trisomy 8 presentation by inflammatory manifestations and its response to thalidomide: two case reports and narrative review.

Zhang X, Zhao Y, Pan Y, Jin J, Fan Z, Yu H Front Pediatr. 2024; 12:1431511.

PMID: 39228438 PMC: 11368770. DOI: 10.3389/fped.2024.1431511.

Chromosomal abnormalities related to fever of unknown origin in a Chinese pediatric cohort and literature review.

Sun B, Yang M, Hou J, Wang W, Ying W, Hui X Orphanet J Rare Dis. 2022; 17(1):292.

PMID: 35897075 PMC: 9327306. DOI: 10.1186/s13023-022-02444-0.

Intestinal Behçet disease associated with myelodysplastic syndrome accompanying trisomy 8 successfully treated with abdominal surgery followed by hematopoietic stem cell transplantation: A case report.

Asano T, Sato S, Furuya M, Takahashi H, Shichishima-Nakamura A, Ohkawara H Medicine (Baltimore). 2019; 98(46):e17979.

PMID: 31725663 PMC: 6867715. DOI: 10.1097/MD.0000000000017979.

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