Myelodysplastic syndromes current treatment algorithm 2018.
Myelodysplastic syndromes current treatment algorithm 2018.
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DOI：
10.1038/s41408-018-0085-4
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发表时间：
2018-05-24
影响因子：
12.8
通讯作者：
Steensma DP
中科院分区：
文献类型：
--
作者：
Steensma DP
骨髓增生异常综合征（MDS）包括一组克隆性骨髓肿瘤，其特征在于由于无效造血、异常血液和骨髓细胞形态导致的血细胞减少，以及克隆性演变和进展为急性髓性白血病（AML）的风险。由于MDS患者的结局是异质性的，因此使用修订的国际预后评分系统（IPSS-R）等工具进行个体风险分层在管理患者时非常重要，包括选择异基因造血干细胞移植（ASCT）的候选人，这是MDS唯一潜在的治愈性治疗方法。IPSS-R可以通过分子遗传学检测进行补充，因为某些基因突变（如TP 53）会影响独立于临床病理学变量的风险。对于有症状性贫血的低风险患者，使用红细胞生成刺激剂（ESA）或来那度胺（特别是染色体5 q缺失的患者）治疗可以改善症状。一些风险较低的患者可能是免疫抑制治疗、血小板生成刺激剂或DNA低甲基化剂（HMA;阿扎胞苷或地西他滨）的候选者。在高风险患者中，移植候选人应尽快接受ASCT，HMA可作为移植的桥梁。非移植候选人应开始HMA治疗，如果耐受，继续治疗，直到疾病进展。在所有人群中，根据需要进行输血和抗菌药物的支持性护理仍然很重要。
Myelodysplastic syndromes (MDS) include a group of clonal myeloid neoplasms characterized by cytopenias due to ineffective hematopoiesis, abnormal blood and marrow cell morphology, and a risk of clonal evolution and progression to acute myeloid leukemia (AML). Because outcomes for patients with MDS are heterogeneous, individual risk stratification using tools such as the revised International Prognostic Scoring System (IPSS-R) is important in managing patients—including selecting candidates for allogeneic hematopoietic stem cell transplantation (ASCT), the only potentially curative therapy for MDS. The IPSS-R can be supplemented by molecular genetic testing, since certain gene mutations such as TP53 influence risk independent of established clinicopathological variables. For lower risk patients with symptomatic anemia, treatment with erythropoiesis-stimulating agents (ESAs) or lenalidomide (especially for those with deletion of chromosome 5q) can ameliorate symptoms. Some lower risk patients may be candidates for immunosuppressive therapy, thrombopoiesis-stimulating agents, or a DNA hypomethylating agent (HMA; azacitidine or decitabine). Among higher risk patients, transplant candidates should undergo ASCT as soon as possible, with HMAs useful as a bridge to transplant. Non-transplant candidates should initiate HMA therapy and continue if tolerated until disease progression. Supportive care with transfusions and antimicrobial drugs as needed remains important in all groups.
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影响因子：
6.2
作者：
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6.2
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影响因子：
12.8
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通讯作者：
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影响因子：
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