Komrokji Rami, Bennett John M
Box 704, James P. Wilmot Cancer Center, Strong Memorial Hospital, 601 Elmwood Avenue, Rochester, NY 14642, USA.
Curr Hematol Rep. 2003 May;2(3):179-85.
Myelodysplastic syndrome is a neoplastic clonal stem cell disorder characterized clinically by bone marrow failure and a tendency to progress to acute myelogenous leukemia. Dysplasia is the pathologic hallmark. The French-American-British classification served as the gold standard for more than two decades. Under the auspice of the World Health Organization, more than 100 hematopathologists in a 3-year cumulative effort issued the new World Health Organization classification, which recognizes multilineage dysplasia. Refractory anemia with excess blasts is divided into two groups. Chronic myelomonocytic leukemia is reclassified under a separate category. Refractory anemia with excess blasts in the transformation group was omitted. Finally, 5q-syndrome is a new subgroup. In addition to the pathologic classification, various prognostic predictors were formatted into scoring systems. Bone marrow blast percentage, cytopenias, and cytogenetics are the backbone for those prognostic models. The International Prognostic Scoring System is a product of pooled data from previous scoring systems and a useful tool to predict survival and acute myelogenous leukemia evolution. This paper discusses the classification and prognosis of myelodysplastic syndromes and their evolution.
骨髓增生异常综合征是一种肿瘤性克隆干细胞疾病，临床特征为骨髓衰竭以及有发展为急性髓系白血病的倾向。发育异常是其病理标志。法美英分类法二十多年来一直是金标准。在世界卫生组织的支持下，100 多位血液病理学家经过 3 年的共同努力发布了新的世界卫生组织分类法，该分类法认可多系发育异常。伴有过多原始细胞的难治性贫血分为两组。慢性粒单核细胞白血病被重新归类到一个单独的类别中。转化型伴有过多原始细胞的难治性贫血被省略。最后，5q 综合征是一个新的亚组。除了病理分类外，各种预后预测指标被整理成评分系统。骨髓原始细胞百分比、血细胞减少和细胞遗传学是这些预后模型的核心。国际预后评分系统是以前评分系统汇总数据的产物，是预测生存和急性髓系白血病演变的有用工具。本文讨论了骨髓增生异常综合征的分类、预后及其演变。
