Department of Hematological Malignancies, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA 02115, USA.
Hematology Am Soc Hematol Educ Program. 2009:645-55. doi: 10.1182/asheducation-2009.1.645.
What is the most sensible way of organizing the disorderly spectrum of acquired marrow failure states collectively known as the myelodysplastic syndromes (MDS)? While the 2008 version of the World Health Organization classification is the current standard, the recent proliferation of MDS prognostic tools illustrates the usefulness of supplemental information for clinical purposes. Many cases of acquired bone marrow failure do not fit cleanly into established MDS categories, yet an alternative diagnosis is not apparent. The term "idiopathic cytopenias of undetermined significance" (ICUS) has been proposed to describe these cases, but there is a paucity of information about the natural history of ICUS. New data on the natural history of MDS associated with a broad range of cytogenetic abnormalities that were not included in the International Prognostic Scoring System (IPSS), as well as the emerging picture of karyotypically occult DNA changes, promise to inform future classifications.
对于紊乱的获得性骨髓衰竭状态谱，即通常所知的骨髓增生异常综合征（MDS），应该如何进行最合理的分类？虽然 2008 年版世界卫生组织（WHO）分类是目前的标准，但最近 MDS 预后工具的大量涌现表明，补充信息对于临床目的是有用的。许多获得性骨髓衰竭的病例不能明确归入既定的 MDS 类别，但又没有明显的其他诊断。因此，有人提出了“意义未明的特发性血细胞减少症”（ICUS）这一术语来描述这些病例，但关于 ICUS 的自然病程的信息却很少。新的数据表明，国际预后积分系统（IPSS）中未包含的广泛细胞遗传学异常与 MDS 相关，以及隐匿性核型 DNA 改变的出现，有望为未来的分类提供信息。
