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
PY - 2019/11/1
Y1 - 2019/11/1
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.
AB - 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.
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U2 - 10.1159/000501019
DO - 10.1159/000501019
M3 - Article
C2 - 31291615
AN - SCOPUS:85071579824
SN - 0001-5792
VL - 142
SP - 253
EP - 256
JO - Acta Haematologica
JF - Acta Haematologica
IS - 4
ER -