Oka Satoko, Ono Kazuo, Nohgawa Masaharu
Division of Hematology, Japanese Red Cross Society Wakayama Medical Center, Wakayama, Japan.
Division of Pathology, Japanese Red Cross Society Wakayama Medical Center, Wakayama, Japan.
Leuk Res Rep. 2020 Mar 12;13:100196. doi: 10.1016/j.lrr.2020.100196. eCollection 2020.
A relationship has been reported between myelodysplastic syndrome (MDS) and autoimmune disease. Behçet's disease is a multisystem inflammatory disorder with mucocutaneous, articular, gastrointestinal, neurological, and vascular manifestations. The co-occurrence of MDS with trisomy 8 and Behçet's-like disease was recently demonstrated. We herein describe a case that shows the relationship between the acquisition of trisomy 8 and occurrence of Behçet's-like disease. Immune dysregulation and altered T-cell hemostasis play an important role in the pathogenesis of Behçet's-like disease and MDS with trisomy 8.
据报道,骨髓增生异常综合征(MDS)与自身免疫性疾病之间存在关联。白塞病是一种多系统炎症性疾病,有皮肤黏膜、关节、胃肠道、神经和血管方面的表现。最近证实了MDS伴8号染色体三体与白塞病样疾病的共存。我们在此描述一例显示8号染色体三体的获得与白塞病样疾病发生之间关系的病例。免疫失调和T细胞稳态改变在白塞病样疾病和伴8号染色体三体的MDS的发病机制中起重要作用。