Behçet's disease (BD) is a systemic vasculitis, while myelodysplastic syndrome (MDS) is a heterogeneous group of clonal hematologic disorders characterized by ineffective hematopoiesis. Some studies suggest a relationship between MDS and BD, especially intestinal BD, and trisomy 8 seems to play an important role in both diseases. There are several reports on patients with BD comorbid with MDS involving trisomy 8 that frequently have intestinal lesions refractory to conventional medical therapies. Tumor necrosis factor (TNF)-α is strongly involved in the pathophysiology of several autoimmune diseases such as rheumatoid arthritis, inflammatory bowel disease, and BD. In addition, TNF-α plays an important role in the pathophysiology of MDS by inhibiting normal hematopoiesis and inducing the programmed cell death of normal total bone marrow cells and normal CD34+ cells. Recent clinical reports demonstrate the favorable effect of TNF-α antagonists in patients with refractory intestinal BD and in those with MDS. We present the case of a patient with intestinal BD and MDS involving trisomy 8 who was successfully treated with adalimumab.

Citations

Citations to this article as recorded by  

• The Expanding Spectrum of Autoinflammatory Diseases
  Kiyoshi Migita, Yuya Fujita, Tomoyuki Asano, Shuzo Sato
  Internal Medicine.2023; 62(1): 43.     CrossRef
• Refractory Intestinal Behçet-Like Disease Associated with Trisomy 8 Myelodysplastic Syndrome Resolved by Parenteral Nutrition
  Ryo Takahashi, Yasuo Matsubara, Satoshi Takahashi, Kazuaki Yokoyama, Lim Lay Ahyoung, Michiko Koga, Hiroyuki Sakamoto, Narikazu Boku, Dai Shida, Hiroshi Yotsuyanagi
  Case Reports in Gastroenterology.2023; 17(1): 287.     CrossRef
• The Expanding Spectrum of Autoinflammatory Diseases
  Kiyoshi Migita, Yuya Fujita, Tomoyuki Asano, Shuzo Sato
  Internal Medicine.2022;[Epub]     CrossRef
• CAISC: A software to integrate copy number variations and single nucleotide mutations for genetic heterogeneity profiling and subclone detection by single-cell RNA sequencing
  Jeerthi Kannan, Liza Mathews, Zhijie Wu, Neal S. Young, Shouguo Gao
  BMC Bioinformatics.2022;[Epub]     CrossRef
• Autoimmune disorders associated with myelodysplastic syndromes: clinical, prognostic and therapeutic implications
  Claudio Fozza, Andrea Murtas, Giovanni Caocci, Giorgio La Nasa
  Leukemia Research.2022; 117: 106856.     CrossRef
• Advances in Management of Intestinal Behçet’s Disease: A Perspective From Gastroenterologists
  Jae Hee Cheon
  Journal of Rheumatic Diseases.2021; 28(1): 4.     CrossRef
• Syndrome de pseudo-Behçet et autres manifestations dysimmunitaires associées aux syndromes myélodysplasiques avec trisomie 8
  N. Wesner, P. Fenaux, V. Jachiet, L. Ades, O. Fain, A. Mekinian
  La Revue de Médecine Interne.2021; 42(3): 170.     CrossRef
• The Clinical Features of Intestinal Disease in Myelodysplastic Syndromes with Trisomy 8
  Yusuke Honzawa
  Nippon Daicho Komonbyo Gakkai Zasshi.2021; 74(10): 594.     CrossRef
• Update on the Treatment of Behcet’s Disease of the Small Bowel with Biologic Agents
  Yehyun Park, Jae Hee Cheon
  Current Gastroenterology Reports.2020;[Epub]     CrossRef
• How to treat myelodysplastic syndrome with clinical features resembling Behçet syndrome: a case-based systematic review
  Umut Yilmaz, Muhlis Cem Ar, Sinem Nihal Esatoglu, Isil Bavunoglu, Yusuf Ziya Erzin, Ali Ibrahim Hatemi, Suleyman Yildirim, Gülen Hatemi, Aykut Ferhat Celik
  Annals of Hematology.2020; 99(6): 1193.     CrossRef
• Evidence-based diagnosis and clinical practice guidelines for intestinal Behçet’s disease 2020 edited by Intractable Diseases, the Health and Labour Sciences Research Grants
  Kenji Watanabe, Satoshi Tanida, Nagamu Inoue, Reiko Kunisaki, Kiyonori Kobayashi, Masakazu Nagahori, Katsuhiro Arai, Motoi Uchino, Kazutaka Koganei, Taku Kobayashi, Mitsuhiro Takeno, Fumiaki Ueno, Takayuki Matsumoto, Nobuhisa Mizuki, Yasuo Suzuki, Tadakaz
  Journal of Gastroenterology.2020; 55(7): 679.     CrossRef
• Sonic hedgehog signaling pathway in Myelodysplastic Syndrome: Abnormal activation and jervine intervention
  YuTing Qin, Ming Jiang, Nilupar Tuerxung, Huan Wang, Fang Zhao, Yin Zhen, Jianping Hao
  Gene.2020; 754: 144881.     CrossRef
• Gastrointestinal Behcet’s-like disease with myelodysplastic neoplasms with trisomy 8: a French case series and literature review
  Nadege Wesner, Louis Drevon, Alexis Guedon, Jean Baptiste Fraison, Benjamin Terrier, Salim Trad, Jean Emmanuel Kahn, Achille Aouba, Jerome Gillard, Matthieu Ponsoye, Thomas Hanslik, Clement Gourguechon, Eric Liozon, Kamel Laribi, Julien Rossignol, Olivier
  Leukemia & Lymphoma.2019; 60(7): 1782.     CrossRef
• Usefulness of Ustekinumab for Treating a Case of Myelodysplastic Syndrome-associated Inflammatory Bowel Disease
  Masashi Kono, Toshiharu Sakurai, Kazuki Okamoto, Tomoyuki Nagai, Yoriaki Komeda, Hiroshi Kashida, Kosuke Minaga, Ken Kamata, Mamoru Takenaka, Satoru Hagiwara, Tomohiro Watanabe, Naoshi Nishida, Eisuke Enoki, Hiroaki Inoue, Itaru Matsumura, Masatoshi Kudo
  Internal Medicine.2019; 58(14): 2029.     CrossRef
• Intestinal Behçet disease associated with myelodysplastic syndrome accompanying trisomy 8 successfully treated with abdominal surgery followed by hematopoietic stem cell transplantation
  Tomoyuki Asano, Shuzo Sato, Makiko Yashiro Furuya, Hiroshi Takahashi, Akiko Shichishima-Nakamura, Hiroshi Ohkawara, Tatsuo Fujiwara, Naohiko Gunji, Choichiro Hashimoto, Tomoyuki Momma, Motonobu Saito, Hiroshi Nakano, Guy Watanabe, Jumpei Temmoku, Yuya Fuj
  Medicine.2019; 98(46): e17979.     CrossRef
• Long-term maintenance of the mucosal healing induced by azacitidine therapy in a patient with intestinal Behçet’s-like disease accompanied with myelodysplastic syndrome involving trisomy 8
  Nahoko Tanaka, Hirotake Sakuraba, Hiroto Hiraga, Ko Mayama, Hidezumi Kikuchi, Dai Kishida, Yui Akemoto, Keisuke Hasui, Shinji Ota, Rina Watanabe, Yasuhisa Murai, Takato Maeda, Kosuke Kamata, Tetsuya Tatsuta, Manabu Sawaya, Daisuke Chinda, Tatsuya Mikami,
  Immunological Medicine.2019; 42(3): 135.     CrossRef
• Updated treatment strategies for intestinal Behçet’s disease
  Yong Eun Park, Jae Hee Cheon
  The Korean Journal of Internal Medicine.2018; 33(1): 1.     CrossRef
• High Incidence of Gastrointestinal Ulceration and Cytogenetic Aberration of Trisomy 8 as Typical Features of Behçet’s Disease Associated with Myelodysplastic Syndrome: A Series of 16 Consecutive Chinese Patients from the Shanghai Behçet’s Disease Database
  Yan Shen, Hai-Fen Ma, Dan Luo, Jian-Fei Cai, Jun Zou, Jian-Long Guan
  BioMed Research International.2018; 2018: 1.     CrossRef
• Anti-Tumor Necrosis Factor Therapy in Intestinal Beh Ccedil;et rsquo;s Disease
  Jihye Park, Jae Hee Cheon
  Gut and Liver.2018; 12(6): 623.     CrossRef
• Update on the Medical Management of Gastrointestinal Behçet’s Disease
  Giuseppe Lopalco, Donato Rigante, Vincenzo Venerito, Claudia Fabiani, Rossella Franceschini, Michele Barone, Giovanni Lapadula, Mauro Galeazzi, Bruno Frediani, Florenzo Iannone, Luca Cantarini
  Mediators of Inflammation.2017; 2017: 1.     CrossRef
• Risk factors and outcomes of acute lower gastrointestinal bleeding in intestinal Behçet’s disease
  Jihye Park, Jae Hee Cheon, Yong Eun Park, Yoon Jee Lee, Hyun Jung Lee, Soo Jung Park, Sung Pil Hong, Tae Il Kim, Won Ho Kim
  International Journal of Colorectal Disease.2017; 32(5): 745.     CrossRef
• 18F-FDG PET/CT Findings in Two Cases With Myelodysplastic Syndrome Accompanied by Behçet’s Disease
  Kimiteru Ito, Kazuo Kubota
  Clinical Nuclear Medicine.2016; 41(8): e392.     CrossRef
• Multiple colonic ulcers associated with trisomy 8: serial changes in colonoscopic findings
  Shunichi Yanai, Shotaro Nakamura, Keisuke Kawasaki, Shigeki Ito, Tamotsu Sugai, Takayuki Matsumoto
  Clinical Journal of Gastroenterology.2016; 9(5): 298.     CrossRef
• Acute Monocytic Leukemia Masquerading Behçet’s Disease-Like Illness at Onset in an Elderly Female
  Shigeru Koba, Toshio Sekioka, Sorou Takeda, Aya Miyagawa-Hayashino, Keisuke Nishimura, Shinsaku Imashuku
  Case Reports in Hematology.2016; 2016: 1.     CrossRef