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Intestinal Behçet's Disease with Primary Myelofibrosis Involving Trisomy 8.
Acta Haematol 2019; :1-4AH

Abstract

Behçet's disease (BD) is a disorder characterized by systemic inflammation of multiple organs, including the intestines. Several studies have reported a relationship between myelodysplastic syndrome and BD, and trisomy 8 was frequently seen, especially in intestinal BD. However, the association of BD with primary myelofibrosis (PMF) has not been well documented. A 58-year-old Japanese female was diagnosed with PMF in 2014. The symptoms of PMF resolved with ruxolitinib. However, she developed fever and intestinal perforation due to multiple ulcers in the terminal ileum in 2017. Intestinal perforation recurred 1 month later, and the dose of ruxolitinib was tapered. After discontinuation of ruxolitinib, she presented with recurrent oral aphthous ulcers and uveitis. Subsequently, intestinal perforation recurred, and she was diagnosed with intestinal BD. Trisomy 8 was identified in her peripheral blood. She underwent steroid therapy, azathioprine, and infliximab. This case suggests relationships between PMF, trisomy 8, and BD.

Authors+Show Affiliations

Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan, mshira@med.kyushu-u.ac.jp.Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.Department of Anatomic Pathology, Pathological Sciences, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.Department of Anatomic Pathology, Pathological Sciences, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. Department of Molecular Endocrinology and Metabolism, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

31291615

Citation

Narazaki, Taisuke, et al. "Intestinal Behçet's Disease With Primary Myelofibrosis Involving Trisomy 8." Acta Haematologica, 2019, pp. 1-4.
Narazaki T, Shiratsuchi M, Tsuda M, et al. Intestinal Behçet's Disease with Primary Myelofibrosis Involving Trisomy 8. Acta Haematol. 2019.
Narazaki, T., Shiratsuchi, M., Tsuda, M., Tsukamoto, Y., Muta, H., Masuda, T., ... Ogawa, Y. (2019). Intestinal Behçet's Disease with Primary Myelofibrosis Involving Trisomy 8. Acta Haematologica, pp. 1-4. doi:10.1159/000501019.
Narazaki T, et al. Intestinal Behçet's Disease With Primary Myelofibrosis Involving Trisomy 8. Acta Haematol. 2019 Jul 10;1-4. PubMed PMID: 31291615.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Intestinal Behçet's Disease with Primary Myelofibrosis Involving Trisomy 8. AU - Narazaki,Taisuke, AU - Shiratsuchi,Motoaki, AU - Tsuda,Mariko, AU - Tsukamoto,Yasuhiro, AU - Muta,Hiroki, AU - Masuda,Toru, AU - Kimura,Daisaku, AU - Takamatsu,Akiko, AU - Nakanishi,Ryota, AU - Oki,Eiji, AU - Fujiwara,Minako, AU - Oda,Yoshinao, AU - Nakashima,Yasuhiro, AU - Ogawa,Yoshihiro, Y1 - 2019/07/10/ PY - 2019/02/07/received PY - 2019/05/15/accepted PY - 2019/7/11/entrez KW - Behçet’s disease KW - Infliximab KW - Intestinal ulcer KW - Primary myelofibrosis KW - Trisomy 8 SP - 1 EP - 4 JF - Acta haematologica JO - Acta Haematol. N2 - Behçet's disease (BD) is a disorder characterized by systemic inflammation of multiple organs, including the intestines. Several studies have reported a relationship between myelodysplastic syndrome and BD, and trisomy 8 was frequently seen, especially in intestinal BD. However, the association of BD with primary myelofibrosis (PMF) has not been well documented. A 58-year-old Japanese female was diagnosed with PMF in 2014. The symptoms of PMF resolved with ruxolitinib. However, she developed fever and intestinal perforation due to multiple ulcers in the terminal ileum in 2017. Intestinal perforation recurred 1 month later, and the dose of ruxolitinib was tapered. After discontinuation of ruxolitinib, she presented with recurrent oral aphthous ulcers and uveitis. Subsequently, intestinal perforation recurred, and she was diagnosed with intestinal BD. Trisomy 8 was identified in her peripheral blood. She underwent steroid therapy, azathioprine, and infliximab. This case suggests relationships between PMF, trisomy 8, and BD. SN - 1421-9662 UR - https://www.unboundmedicine.com/medline/citation/31291615/Intestinal_Behçet's_Disease_with_Primary_Myelofibrosis_Involving_Trisomy_8 L2 - https://www.karger.com?DOI=10.1159/000501019 DB - PRIME DP - Unbound Medicine ER -