Aul C, Giagounidis A, Germing U, Ganser A
Medizinische Klinik II (Hämatologie, Onkologie und Immunologie), St. Johannes-Hospital Duisburg, An der Abtei 7-11, 47166 Duisburg, Germany.
Ann Hematol. 2002 Sep;81(9):485-97. doi: 10.1007/s00277-002-0530-z. Epub 2002 Sep 24.
One of the hallmarks of myelodysplastic syndromes (MDS) is their prognostic heterogeneity which complicates decision making regarding treatment for individual patients. The French-American-British (FAB) classification provides significant prognostic information, but carries the disadvantage of arbitrary demarcation of subgroups and overemphasis of morphological findings. In addition, there is considerable variation in survival and risk of acute myeloblastic leukemia (AML) development even within defined FAB subgroups, particularly in patients with refractory anemia with ring sideroblasts (RARS) and chronic myelomonocytic leukemia (CMML). Over the last 2 decades, several research groups have tried to identify additional clinical, hematological, and cell biological parameters in order to more accurately predict the natural course of MDS. These investigations have clarified that the number and extent of peripheral blood cytopenias, the bone marrow blast count, and the cytogenetic pattern are the most powerful prognostic indicators in MDS. Recent efforts have been directed at constructing prognostic scoring systems. These scoring systems try to enhance the predictive power by combining several features of the disease, which have proved their independent prognostic weight on multivariate analysis. The International MDS Risk Analysis Workshop substantially advanced the prognostic categorization of MDS patients by proposing a new scoring system (International Prognosis Scoring System, IPSS) that can be successfully applied to risk assessment of newly diagnosed patients and will likely prove useful for the design and analysis of therapeutic trials in MDS.
骨髓增生异常综合征（MDS）的一个显著特征是其预后的异质性，这使得针对个体患者的治疗决策变得复杂。法国-美国-英国（FAB）分类提供了重要的预后信息，但存在亚组划分随意以及过度强调形态学发现的缺点。此外，即使在明确的FAB亚组内，生存情况和急性髓系白血病（AML）发生风险也存在相当大的差异，尤其是在伴有环形铁粒幼细胞的难治性贫血（RARS）和慢性粒-单核细胞白血病（CMML）患者中。在过去20年里，多个研究小组试图确定其他临床、血液学和细胞生物学参数，以便更准确地预测MDS的自然病程。这些研究表明，外周血细胞减少的数量和程度、骨髓原始细胞计数以及细胞遗传学模式是MDS中最有力的预后指标。最近的努力方向是构建预后评分系统。这些评分系统试图通过结合疾病的多个特征来提高预测能力，这些特征在多变量分析中已证明具有独立的预后权重。国际MDS风险分析研讨会通过提出一种新的评分系统（国际预后评分系统，IPSS），极大地推进了MDS患者的预后分类，该系统可成功应用于新诊断患者的风险评估，并且可能对MDS治疗试验的设计和分析有用。
