Hofmann Wolf K, Koeffler H Phillip
Division of Hematology/Oncology, Cedars-Sinai Research Institute, UCLA School of Medicine, Los Angeles, California, USA.
Int J Hematol. 2002 Aug;76 Suppl 2:222-7. doi: 10.1007/BF03165121.
Myelodysplastic syndromes (MDS) are characterized by peripheral cytopenias in combination with a hyperplastic bone marrow. During the last 15 years, important progress has been made in the understanding of the biology and prognosis of myelodysplastic syndromes. The classification according to the World Health Organization (WHO) includes mainly morphological criteria and is supplemented by the International Prognostic Scoring System (IPSS) which takes cytogenetical changes into consideration when determining the prognosis of MDS. Also MDS after radiotherapy, chemotherapy or chemical exposure must be distinguished from primary MDS. The underlying mechanisms in primary MDS have not yet been established but it is a multistep alteration to the hematopoietic stem cells that include genes involved in cell cycle control, mitotic checkpoints as well as growth factor receptors, secondary signal proteins and transcription factors which gives the cell a growth advantage over its normal counterpart.
骨髓增生异常综合征（MDS）的特征是外周血细胞减少伴骨髓增生。在过去15年中，对骨髓增生异常综合征的生物学特性和预后的认识取得了重要进展。世界卫生组织（WHO）的分类主要依据形态学标准，并辅以国际预后评分系统（IPSS），该系统在确定MDS的预后时会考虑细胞遗传学变化。此外，放疗、化疗或化学物质暴露后发生的MDS必须与原发性MDS区分开来。原发性MDS的潜在机制尚未明确，但它是造血干细胞的多步骤改变，涉及细胞周期调控、有丝分裂检查点以及生长因子受体、二级信号蛋白和转录因子等基因，这些基因赋予细胞相对于正常细胞的生长优势。
