摘要
骨髓增生异常综合征是克隆性造血组织肿瘤,表现为慢性血细胞减少症伴细胞形态发育异常,易进展为骨髓衰竭或急性髓系白血病。贝赫切特综合征是一类特殊的系统性血管炎,主要表现为复发性口腔溃疡、皮肤损害、眼部炎症等。近年来,骨髓增生异常综合征合并贝赫切特综合征的病例报道增多,提示两者之间存在潜在的病理联系。免疫细胞的异常激活、细胞因子的异常分泌以及细胞遗传学改变等可能在骨髓增生异常综合征合并贝赫切特综合征的发病机制中起重要作用。目前,骨髓增生异常综合征合并贝赫切特综合征的治疗策略主要基于个体化原则,包括免疫抑制治疗、细胞毒性药物治疗、靶向治疗及造血干细胞移植等。然而,由于病例报道较少及机制研究不足,治疗方案的选择仍需探讨。本文综述了骨髓增生异常综合征与贝赫切特综合征的关联性及其机制,总结了相关治疗进展,以期为该类患者的临床管理和相关机制的进一步研究提供参考。
Myelodysplastic syndromes(MDS)are clonal hematopoietic neoplasms characterized by chronic cytopenias and abnormal cell morphology,with a propensity of progressing to bone marrow failure or acute myeloid leukemia.Behçet’s syndrome is a systemic vasculitis characterized by recurrent oral ulcers,skin lesions,and ocular inflammation.In recent years,an increasing number of clinical cases with coexistence of MDS and Behçet’s syndrome have been reported,suggesting a potential pathological relationship between these conditions.Abnormal immune cell activation,dysregulated cytokine secretion,and cytogenetic alterations are thought to play critical roles in the pathogenesis of MDS combined with Behçet’s syndrome.Currently,treatment strategies for MDS combined with Behçet’s syndrome are primarily individualized and include immunosuppressive therapy,cytotoxic drug therapy,targeted therapy,and hematopoietic stem cell transplantation.However,due to the limited number of case reports and insufficient research on the underlying mechanisms,selecting appropriate treatment options remains challenging.This article reviews the pathogenesis and interrelationships of MDS combined with Behçet’s syndrome and summarizes recent advancements in treatment strategies,providing a reference for clinical management and further researches on related mechanisms.
作者
付慧
余建
FU Hui;YU Jian(Department of Hematology and Oncology,Taizhou Hospital of Zhejiang Province,Taizhou 317000,Zhejiang Province,China;Bone Marrow Transplantation Center,the First Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou 310003,China)
出处
《浙江大学学报(医学版)》
北大核心
2025年第1期131-139,共9页
Journal of Zhejiang University(Medical Sciences)
基金
国家自然科学基金(82270221)。
作者简介
第一作者:付慧,医师,主要从事血液病临床和基础研究,E-mail:fhdoct@163.com,ORCID:0009-0003-0454-1656;通信作者:余建,主任医师,硕士生导师,主要从事血液病临床和基础研究及造血干细胞移植,E-mail:zjuyujian@zju.edu.cn,ORCID:0000-0002-8704-1571。
