骨髓增生异常/骨髓增殖性肿瘤伴环形铁粒幼细胞和血小板增多的临床特点及治疗进展  

Clinical characteristics and treatment of myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis

作　　者：金炜 Jin Wei(Department of Hematology,Kunshan First People's Hospital,Kunshan 215300,China)

机构地区：[1]昆山市第一人民医院血液科,江苏昆山215300

出　　处：《中华临床医师杂志（电子版）》2024年第1期101-104,共4页Chinese Journal of Clinicians(Electronic Edition)

摘　　要：骨髓增生异常/骨髓增殖性肿瘤伴环状铁粒幼细胞和血小板增多(MDS/MPN-RS-T)是WHO在2016年最新版MDS/MPN的分类标准中新增加的分型。是一类同时具有伴环形铁粒幼细胞增多的骨髓增生异常综合征(MDS-RS)的病态造血及巨核细胞异常克隆性增殖导致血小板增多的临床特点的疾病。因其发病率极低,相关文献报道较少,临床工作中十分容易漏诊及误诊,即使确诊后也无系统的治疗方案,本文通过参考国内外针对这类疾病诊断与治疗的相关报道进行分析总结,拟提高临床工作中对这类疾病的认识。In 2016,the World Health Organization updated the classification criteria for myelodysplastic syndrome(MDS)/myeloproliferative neoplasm(MPN),which added the classification of myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis(MDS/MPN-RS-T).MDS/MPN-RS-T has the dual characteristics of myelodysplasia and myeloproliferation at the same time.Because its incidence rate is extremely low and there are few relevant literature reports,it is very prone to missed diagnosis and misdiagnosis in clinical work,and there is no systematic treatment plan even after diagnosis.This article analyzes and summarizes the relevant reports on the diagnosis and treatment of MDS/MPN-RS-T at home and abroad,with an aim to improve the understanding of this rare disease among clinicians.

关 键 词：骨髓增生异常综合征/骨髓增殖性肿瘤 环状铁粒幼细胞 血小板增多 临床特点 治疗进展 

分 类 号：R733.3[医药卫生—肿瘤]