JSH practical guidelines for hematological malignancies, 2018: I. Leukemia-6 myelodysplastic syndromes (MDS)
JSH practical guidelines for hematological malignancies, 2018: I. Leukemia-6 myelodysplastic syndromes (MDS)
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2018 年 JSH 血液系统恶性肿瘤实用指南：I. 白血病 6 型骨髓增生异常综合征 (MDS)
DOI：
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发表时间：
2020
影响因子：
2.1
通讯作者：
Y. Miyazaki
中科院分区：
文献类型：
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作者：
Y. Miyazaki
骨髓增生异常综合征（MDS）是以造血细胞异常增殖和凋亡为特征的骨髓肿瘤，认为是由未成熟造血细胞异常引起的[1，2]。MDS的概念首次在1982年的法国-美国-英国（FAB）分类中描述[3]，目前，MDS使用2001年[4]和2008年[5] WHO分类以及2017年修订的WHO分类[6]进行分类。然而，这些也与FAB分类结合使用。MDS的特征在于单系或多系血细胞减少、形态发育不良、无效造血和转化为急性白血病的风险，并且被认为不是单一疾病，而是包括多种疾病的一组综合征。因此，仅将其分类为目前定义的亚型不足以做出临床决策。超过一半的MDS患者具有细胞遗传学异常，并且未分化造血细胞的遗传异常被认为参与MDS的发病机制。越来越多的新的遗传异常无法检测到的细胞遗传学检测已被确定，它被认为是主要的几乎都是已知的这一点[7]，但其意义的进一步调查是必要的。MDS通常使用WHO分类（2017）基于血细胞减少、外周血和骨髓中原始细胞百分比、造血细胞发育不良和细胞遗传学异常进行诊断。在某些情况下，还参考使用FAB分类的诊断。MDS的概念与各种其他血液学疾病有共同的边界，即使在今天，其诊断（包括临床病程观察和排除其他疾病）仍然严重依赖于形态学评估[1，2]。在达到明确诊断后，根据用于诊断的血液学结果、骨髓结果和细胞遗传学异常预测预后，并制定治疗计划。预后主要通过与血细胞减少相关的事件来预测（例如，感染和出血）和白血病转化，但由于MDS在老年人中的高患病率，患者特征如伴随疾病也对预后有很大影响。目前，异基因造血干细胞移植（HSCT）仍然是唯一的治愈性疗法，但由于患者人群高龄等因素，只有部分患者能从异基因移植中获益。然而，最近，MDS的新药已经开发出来，治疗方面也取得了其他新的进展。
Myelodysplastic syndromes (MDS) are myeloid neoplasms characterized by abnormal proliferation and apoptosis of hematopoietic cells that are believed to be caused by abnormalities in immature hematopoietic cells [1, 2]. The concept of MDS was first described in the French-American-British (FAB) classification in 1982 [3], and currently, MDS is classified using the 2001 [4] and 2008 [5] WHO classifications, as well as the 2017 revised WHO classification [6]. However, these are also used in combination with the FAB classification. MDS is characterized by unilineage or multilineage cytopenia, morphological dysplasia, ineffective hematopoiesis, and risk for transformation to acute leukemia, and is considered to be not a single disease but rather a group of syndromes comprising multiple diseases. Therefore, classification into presently defined subtypes alone is insufficient for making clinical decisions. More than half of patients with MDS have cytogenetic abnormalities, and genetic abnormalities in undifferentiated hematopoietic cells are believed to be involved in the pathogenesis of MDS. More and more new genetic abnormalities undetectable by cytogenetic testing have been identified and it is believed that the major ones are almost all known by this point [7], but further investigation of their significance is warranted. MDS is typically diagnosed using the WHO classification (2017) on the basis of cytopenia, percentages of blasts in peripheral blood and bone marrow, hematopoietic cell dysplasia, and cytogenetic abnormalities. In some cases, diagnosis using the FAB classification is also referenced. The concept of MDS shares boundaries with various other hematologic diseases, and their diagnosis, including clinical course observation and exclusion of other diseases, continues to rely heavily on morphological assessments even today [1, 2]. After a definitive diagnosis is reached, a prognosis is predicted on the basis of the hematological findings, bone marrow findings, and cytogenetic abnormalities used to make the diagnosis, and a treatment plan is formulated. The prognosis is primarily predicted by events related to cytopenia (e.g., infection and hemorrhage) and leukemic transformation, but patient characteristics such as concomitant diseases also have a large impact on prognosis due to the high prevalence of MDS in elderly adults. At present, allogeneic hematopoietic stem cell transplantation (HSCT) remains the only curative therapy, but due to factors such as the advanced age of the patient population, only some patients can benefit from allogeneic transplantation. Recently, however, new drugs for MDS have been developed and other new advances in treatment have been made.
与常规护理方案相比，阿扎西丁氨酸的疗效在治疗高风险的髓质发育异常综合征中：一项随机，开放标签的III期研究。
影响因子：
51.1
作者：
Fenaux, Pierre;Mufti, Ghulam J.;Hellstrom-Lindberg, Eva;Santini, Valeria;Finelli, Carlo;Giagounidis, Aristoteles;Schoch, Robert;Gattermann, Norbert;Sanz, Guillermo;List, Alan;Gore, Steven D.;Seymour, John F.;Bennett, John M.;Byrd, John;Backstrom, Jay;Zimmerman, Linda;McKenzie, David;Beach, C. L.;Silverman, Lewis R.
通讯作者：
Silverman, Lewis R.
影响因子：
45.3
作者：
Silverman, LR;Demakos, EP;Holland, JF
通讯作者：
Holland, JF