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    5q-综合征发病机制及治疗研究进展

    Research development of 5qsyndrome

    • 摘要: 5q综合征是一种造血干细胞疾病,90%以上的细胞来源于恶性克隆,存在于约10-20%的MDS患者中,5号染色体缺失区域所包含的基因缺失是5q综合征发生的基础,其主要临床表现为巨细胞性贫血,分叶减少的巨核细胞增多,血小板升高或正常,随着临床医生对新病人的诊断评估,我们对5q综合征有了不同的认识,病人在临床实践中出现了一些不同的临床表现;雷那度胺被认为是治疗低危输血依赖的5q综合征的标准,由于其存在的副作用,而且不是所有的5号染色体缺失病人对雷那度胺都有反应。因此,需要新的治疗方案对5号染色体缺失病人进行治疗,现就5q综合征的诊断和治疗进展做一综述。

       

      Abstract: 5 q syndrome is a disease of hematopoietic stem cells with more than 90% of the cells from malignant clones, exist in approximately 10-20% of the patients with MDS.The lack of chromosome 5 area contains gene is the basis of 5 q syndrome occurs, Its main clinical manifestation is a macrocytic anemia, hypolobulated megakaryocytes, normal to elevated platelet count,As clinicians diagnostic evaluation for new patients, we have a different understanding in 5 q syndrome,some unusual clinical situations have emerged in our clinical practice. Lenalidomide is considered to be the standardbe to treat low-risk 5 q syndrome, because of its side effects and not all patients have reaction on the Lenalidomide. Therefore, we need new treatments for lack of chromosome 5 patients , now we narrate the 5 q syndrome diagnosis and treatment progress.

       

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