Lawrence Louann W
Department of Clinical Laboratory Science, Louisiana State University Health Sciences Center, New Orleans, LA 70112, USA.
Clin Lab Sci. 2004 Summer;17(3):178-86.
Refractory anemia is a component of each of the myelodysplastic syndromes (MDSs). MDSs are acquired pluripotent stem cell disorders leading to one or more peripheral blood cytopenias with dysplasia in the peripheral blood and bone marrow. MDS and aplastic anemia are sometimes grouped as bone marrow failure disorders because patients present with similar peripheral blood pictures. The bone marrow in MDS is generally hypercellular, due to ineffective hematopoiesis, in contrast to the hypocellular bone marrow of aplastic anemia. MDS is more common in the elderly, differing from aplastic anemia that affects all ages. The characteristics of each of the subgroups of the MDS using the World Health Organization (WHO) classification are described. Cytogenetic analysis provides a useful part of disease diagnosis in this new classification system. There is no successful treatment for MDS other than hematopoietic stem cell transplantation which is usually recommended for patients under age 50. A prognostic scoring system has been developed to help predict the severity of disease and guide treatment. Approximately 10% to 40% of MDS cases terminate in acute leukemia. Current treatment consists mostly of supportive measures; however several new therapies are being explored.
难治性贫血是各型骨髓增生异常综合征（MDS）的一个组成部分。MDS是获得性多能干细胞疾病，可导致一种或多种外周血细胞减少，并伴有外周血和骨髓发育异常。MDS和再生障碍性贫血有时被归为骨髓衰竭性疾病，因为患者的外周血象相似。与再生障碍性贫血的骨髓细胞减少不同，MDS的骨髓通常因造血无效而细胞增多。MDS在老年人中更常见，这与影响所有年龄段的再生障碍性贫血不同。本文描述了采用世界卫生组织（WHO）分类法的MDS各亚组的特征。细胞遗传学分析在这个新的分类系统中为疾病诊断提供了有用的依据。除造血干细胞移植外，MDS尚无成功的治疗方法，造血干细胞移植通常推荐用于50岁以下的患者。已经开发了一种预后评分系统来帮助预测疾病的严重程度并指导治疗。大约10%至40%的MDS病例会发展为急性白血病。目前的治疗主要包括支持性措施；然而，正在探索几种新的治疗方法。
