Goździk J, Lewandowski K, Hansz J
Kliniki Hematologii Instytutu Chorób Wewnetrznych Akademii Medycznej, Poznaniu.
Acta Haematol Pol. 1995;26(2):129-38.
Myelodysplastic syndromes (MDS) comprise a heterogenous group of closely related, acquired stem cell disorders. Various patterns of clinical evolution have been observed in patients with different subtypes according to the FAB (French-American-British) criteria. The marked differences in clinical outcome among MDS patients have encouraged us to search the alternative variables for predicting leukemic transformation and survival. During the past 10 years different prognostic scoring systems based on age combined with blood and bone marrow parameters have been described. This has enabled the identification of patients with better and worse prognosis among different MDS types. Additionally, studies of cytogenic patterns in MDS patients and widespread availability of bone marrow histological specimens extended the possibility of prognosis in this disease. Similarly, the in vitro culture results of hematopoietic cells using growth and differentiation factors have given very promising results. The high prevalence of RAS mutation in patients with MDS has been defined, but its clinical usefulness is under discussion.
骨髓增生异常综合征(MDS)是一组密切相关的、后天获得性干细胞疾病,具有异质性。根据法国-美国-英国(FAB)标准,不同亚型的患者呈现出各种临床演变模式。MDS患者临床结局的显著差异促使我们寻找预测白血病转化和生存的替代变量。在过去10年中,已经描述了基于年龄并结合血液和骨髓参数的不同预后评分系统。这使得能够在不同的MDS类型中识别出预后较好和较差的患者。此外,对MDS患者细胞遗传学模式的研究以及骨髓组织学标本的广泛可得性扩展了该疾病预后评估的可能性。同样,使用生长和分化因子进行造血细胞的体外培养结果也给出了非常有前景的结果。已经明确了MDS患者中RAS突变的高发生率,但其临床实用性仍在讨论中。