First Department of Internal Medicine, Faculty of Medicine, University of Yamanashi, Japan.
Department of Pathology, Faculty of Medicine, University of Yamanashi, Japan.
Intern Med. 2022 Jun 1;61(11):1713-1719. doi: 10.2169/internalmedicine.8395-21. Epub 2021 Nov 6.
Behçet's disease (BD) is a multisystem inflammatory disease of unknown origin. It rarely but occasionally occurs together with myelodysplastic syndrome and primary myelofibrosis. Trisomy 8 is one of the most common cytogenetic abnormalities in myeloid neoplasms; however, the association of BD with polycythemia vera (PV) and trisomy 8 has not been reported. A 70-year-old woman, diagnosed with PV and treated with hydroxyurea, had bloody stool due to multiple ulcers in the ileocecal region. Considering the lack of a response to treatment and other features, we suspected complication with intestinal Behçet's-like disease. Our case suggests relationships among BD, trisomy 8, and PV.
贝切特病(BD)是一种病因不明的多系统炎症性疾病。它很少但偶尔会与骨髓增生异常综合征和原发性骨髓纤维化同时发生。三体 8 是髓系肿瘤中最常见的细胞遗传学异常之一;然而,BD 与真性红细胞增多症(PV)和三体 8 的关联尚未报道。一位 70 岁女性,诊断为 PV 并接受羟基脲治疗,因回盲部多发性溃疡而出现血便。考虑到缺乏治疗反应和其他特征,我们怀疑并发肠道贝切特样疾病。我们的病例提示 BD、三体 8 和 PV 之间存在关系。