- Inflammatory bowel diseases
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Long-term outcome after surgery in a patient with intestinal Behçet’s disease complicated by myelodysplastic syndrome and trisomy 8
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Yuki Mori, Fumihiko Iwamoto, Yasuaki Ishida, Toru Kuno, Shoji Kobayashi, Takashi Yoshida, Tatsuya Yamaguchi, Tadashi Sato, Makoto Sudo, Daisuke Ichikawa, Nobuyuki Enomoto
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Intest Res 2020;18(4):469-475. Published online May 12, 2020
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DOI: https://doi.org/10.5217/ir.2019.09141
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 Abstract
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- Behçet’s disease (BD) is a multisystem inflammatory disease of unknown origin. Rarely, BD occurs together with myelodysplastic syndrome (MDS). Interestingly, it is speculated that these are not simple coexistence but that the etiology of intestinal BD is at least partly derived from MDS itself. Furthermore, there is a relationship between MDS in patients with intestinal BD and trisomy 8. Immunosuppressive agents alone are insufficient to control MDS-associated BD, and many of these patients die of infection or hemorrhage. Surgery is considered for intestinal BD patients who are unresponsive to medical treatment or those with bowel complications such as perforation or persistent bleeding. We report a case of intestinal BD associated with MDS and trisomy 8. The patient was unresponsive to oral steroids and immunosuppressive treatment; the patient improved by surgical repair of a bowel perforation. Five years after the surgery, the patient is free of recurrence and not on medication. Our experience suggests that surgery may provide an effective therapeutic option for the treatment of MDS-related BD.
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Citations
Citations to this article as recorded by  - Optimal Treatment Approaches to Intestinal Behçet's Disease Complicated by Myelodysplastic Syndrome: The KASID and KSBD Multicenter Study
Jung-Bin Park, So Jung Han, Seung Bum Lee, Dong Hyun Kim, Jae Hee Cheon, Sung Wook Hwang, Byong Duk Ye, Suk-Kyun Yang, Soo Jung Park, Sang Hyoung Park
Yonsei Medical Journal.2024; 65(5): 265. CrossRef - Long‐term clinical outcomes of intestinal Behçet's disease: A 30‐year cohort study at a tertiary hospital in South Korea
Jihye Park, Soo Jung Park, Jae Jun Park, Tae Il Kim, Jae Hee Cheon
Journal of Gastroenterology and Hepatology.2023; 38(3): 386. CrossRef - Interplay between chronic inflammation and clonal haematopoiesis of indeterminate potential in Behçet’s disease
Jihye Park, Hongyul An, Jiwoo Lim, I Seul Park, Mi Hyun Kim, Ji Hyung Kim, Seung Won Kim, Young Il Koh, Eun Young Lee, Jae Hee Cheon
Arthritis Research & Therapy.2023;[Epub] CrossRef - Real-world effectiveness and safety of adalimumab in Korean patients with intestinal Behcet’s disease: a Korean Association for the Study of Intestinal Diseases (KASID) multicenter study
Seung Bum Lee, Hee Seung Hong, Chang Kyun Lee, Bo-In Lee, Sol Kim, Seong-Joon Koh, Hosun Yu, Jung-Bin Park, Sung Wook Hwang, Byong Duk Ye, Suk-Kyun Yang, Sang Hyoung Park
The Korean Journal of Internal Medicine.2023; 38(5): 661. CrossRef - Effectiveness and safety of adalimumab in patients with intestinal Behçet’s disease: a real-world prospective observational study in South Korea
Jongwook Yu, Sung Jae Shin, Yune-Jung Park, Hyung Wook Kim, Bo-In Lee, Byong Duk Ye, Geun-Tae Kim, Sung Kook Kim, Joo Sung Kim, Young-Ho Kim, Seonjeong Jeong, Jae Hee Cheon
BMC Gastroenterology.2023;[Epub] CrossRef - Trisomy 8-positive Polycythemia Vera Complicated with Intestinal Behçet's-like Disease: A New Perspective for a Clinical Approach
Yuki Mori, Fumihiko Iwamoto, Toru Kuno, Shoji Kobayashi, Takashi Yoshida, Tatsuya Yamaguchi, Shinichi Takano, Tetsuo Kondo, Keita Kirito, Nobuyuki Enomoto
Internal Medicine.2022; 61(11): 1713. CrossRef - Clinical Course of Patients with Intestinal Behçet’s Disease According to Consensus-Based Diagnostic Categories
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Gut and Liver.2022; 16(5): 746. CrossRef
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