Department of Hematology Oncology, Fondazione IRCCS Policlinico San Matteo and University of Pavia Medical School, Italy.
Semin Oncol. 2011 Oct;38(5):627-34. doi: 10.1053/j.seminoncol.2011.04.007.
Myelodysplastic syndromes (MDS) are myeloid neoplasms characterized by dysplasia in one or more cell lines and increased risk of development of acute myeloid leukemia (AML). The current diagnostic approach to MDS includes peripheral blood and bone marrow morphology to evaluate abnormalities of peripheral blood cells and hematopoietic precursors; bone marrow biopsy to assess marrow cellularity, fibrosis, and topography; and cytogenetics to identify non-random chromosomal abnormalities. The 2008 World Health Organization (WHO) classification currently provides the best diagnostic approach to MDS and also has considerable prognostic relevance. The WHO classification-based prognostic scoring system (WPSS) is able to classify MDS patients into five risk groups showing different survivals and probabilities of leukemic evolution. The WPSS is able to predict survival and leukemia progression at any time during follow-up, and can therefore be used for implementing risk-adapted treatment strategies in patients with primary MDS. Since comorbidities have a significant impact on the outcome of patients with MDS, accounting for both disease status and comorbid conditions considerably improves risk stratification.
骨髓增生异常综合征(MDS)是一种髓系肿瘤,其特征为一条或多条细胞谱系发育异常,并伴有急性髓系白血病(AML)的发病风险增加。目前 MDS 的诊断方法包括外周血和骨髓形态学,以评估外周血细胞和造血前体细胞的异常;骨髓活检以评估骨髓细胞密度、纤维化和拓扑结构;以及细胞遗传学,以识别非随机染色体异常。2008 年世界卫生组织(WHO)分类目前为 MDS 提供了最佳的诊断方法,同时也具有相当大的预后相关性。基于 WHO 分类的预后评分系统(WPSS)能够将 MDS 患者分为五个风险组,显示出不同的生存率和白血病演变的概率。WPSS 能够在随访的任何时间预测生存和白血病进展,因此可用于对原发性 MDS 患者实施风险适应的治疗策略。由于合并症对 MDS 患者的预后有重大影响,因此同时考虑疾病状态和合并症可显著改善风险分层。