骨髓增生异常综合征与免疫相关性肠道疾病的研究进展  

Advances of myelodysplastic syndrome and immune related intestinal diseases

作　　者：穆燕菊(综述) 吴静(综述)[1] 缪应雷(审校)[1] MU Yan-ju;WU Jing;MIAO Ying-lei(Department of Gastroenterology,the First Affiliated Hospital of Kunming Medical College,Kunming 650032,Yunnan,China)

机构地区：[1]昆明医科大学第一附属医院消化内科,昆明650032

出　　处：《医学研究生学报》2021年第1期74-79,共6页Journal of Medical Postgraduates

基　　金：国家自然科学基金(81660100,81670501);云南省卫计委医学领军人才(L-201607)。

摘　　要：骨髓增生异常综合征(MDS)是一组起源于造血干细胞的克隆性疾病,为多因素、多阶段、连续动态的病理过程,其病因尚不明确,遗传畸变和免疫异常在发病中起重要作用。近年来研究发现约15%~25%的MDS与炎症性或自身免疫性疾病相关,其中以白塞病、炎症性肠病(IBD)和乳糜泻较为多见,两者之间可能存在发病机制关联。MDS合并自身免疫性疾病具有临床表现多样、诊断困难、疗效欠佳,致残和致死率高、预后差,中位生存时间明显缩短等特点,给临床带来了巨大挑战。文章就MDS合并白塞病、IBD及乳糜泻的流行病学、临床表现、诊断、治疗及预后等研究现状进行综述。Myelodysplastic syndromes(MDS)is a group of clonal diseases originated from hematopoietic stem cells.It is a multi-factor,multi-stage,continuous and dynamic pathological process.The pathogeny of the disease is not clear,and genetic aberrations and immune disorders play an important role in the pathogenesis.Studies have found that about 15%-25% of MDS is associated with inflammatory or autoimmune diseases recently,of which Behcet’s disease(BD),inflammatory bowel disease(IBD)and Celiac disease are most common,and may be related to their pathogenesis.MDS combined with autoimmune diseases is characterized by diverse clinical manifestations,difficult diagnosis,poor efficacy,disability,high mortality,poor prognosis,and significantly shortened median survival time,which brings great challenges to clinical practice.This article reviews the epidemiology,clinical manifestations,diagnosis,treatment and prognosis of MDS complicated with BD,IBD and Celiac disease.

关 键 词：骨髓增生异常综合征 白塞病 炎症性肠病 乳糜泻 

分 类 号：R551.3[医药卫生—血液循环系统疾病]