Erythroleukemia shares biological features and outcome with myelodysplastic syndromes with excess blasts: a rationale for its inclusion into future classifications of myelodysplastic syndromes
Erythroleukemia shares biological features and outcome with myelodysplastic syndromes with excess blasts: a rationale for its inclusion into future classifications of myelodysplastic syndromes
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DOI：
10.1038/modpathol.2016.146
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发表时间：
2016-12-01
期刊：
影响因子：
7.5
通讯作者：
Florensa, Lourdes
中科院分区：
文献类型：
--
作者：
Calvo, Xavier;Arenillas, Leonor;Florensa, Lourdes
在2008年世界卫生组织(WHO)的分类中，红细胞白血病被认为是一种急性髓系白血病，其定义是骨髓中有50%的红细胞来自非红系细胞，20%的骨髓母细胞来自非红系细胞。红白血病的临床病理特征与骨髓增生异常综合征相似，尤其是以红系为主的骨髓增生异常综合征(50%的骨髓红细胞)。世卫组织即将修订的版本建议取消非红系原始细胞计数规则，并根据原始细胞绝对计数将红白血病患者归入适当的骨髓增生异常综合征类别。我们对初发红白血病患者进行了一项回顾性研究，并将他们的临床生物学特征和预后与初发骨髓增生异常综合征的患者进行了比较，重点是以红系为主的骨髓增生异常综合征。405例红系占优势的骨髓增生异常综合征患者的中位总生存期显著长于57例红系占优势的难治性贫血和59例红系白血病，但红系占优势的难治性贫血和红系白血病之间无显著差异。在>；=50%的骨髓红系细胞和过多的原始细胞的患者中，国际预后评分系统或修订的国际预后评分系统定义的高危核型的存在是主要的预后因素。同样，459例原始细胞过多的难治性贫血的存活率与59例红白血病的存活率几乎相同，与非红系细胞是否有20%的骨髓原始细胞无关。有趣的是，11例原始细胞数较低的红白血病患者的存活率与其他红白血病患者相似，其中有核细胞占总有核细胞的5%~10%。我们的数据表明，新发红白血病属于原始细胞增多的骨髓增生异常综合征，并支持将其纳入未来的骨髓增生异常综合征的分类。
Erythroleukemia was considered an acute myeloid leukemia in the 2008 World Health Organization (WHO) classification and is defined by the presence of >= 50% bone marrow erythroblasts, having = 20% bone marrow myeloblasts from nonerythroid cells. Erythroleukemia shares clinicopathologic features with myelodysplastic syndromes, especially with erythroid-predominant myelodysplastic syndromes (>= 50% bone marrow erythroblasts). The upcoming WHO revision proposes to eliminate the nonerythroid blast cell count rule and to move erythroleukemia patients into the appropriate myelodysplastic syndrome category on the basis of the absolute blast cell count. We conducted a retrospective study of patients with de novo erythroleukemia and compared their clinico-biological features and outcome with those of de novo myelodysplastic syndromes, focusing on erythroid-predominant myelodysplastic syndromes. Median overall survival of 405 erythroid-predominant myelodysplastic syndromes without excess blasts was significantly longer than that observed in 57 erythroid-predominant refractory anemias with excess blasts-1 and in 59 erythroleukemias, but no significant difference was observed between erythroid-predominant refractory anemias with excess blasts-1 and erythroleukemias. In this subset of patients with >= 50% bone marrow erythroblasts and excess blasts, the presence of a high-risk karyotype defined by the International Prognostic Scoring System or by the Revised International Prognostic Scoring System was the main prognostic factor. In the same way, the survival of 459 refractory anemias with excess blasts-2, independently of having >= 20% bone marrow blasts from nonerythroid cells or not, was almost identical to the observed in 59 erythroleukemias. Interestingly, 11 low-blast count erythroleukemias with 5 to < 10% bone marrow blasts from total nucleated cells showed similar survival than the rest of erythroleukemias. Our data suggest that de novo erythroleukemia is in the spectrum of myelodysplastic syndromes with excess blasts and support its inclusion into future classifications of myelodysplastic syndromes.