Prognosis Of

As stated previously, the clinical and prognostic heterogeneity of MDS confers an additional difficulty when planning therapy. Some patients experience prolonged survival and remain symptom-free for many years, whereas others die within a few weeks or months after diagnosis. Overall, the median survival is nearly 19 months and the risk of AML evolution is 35% at 5 years.3a Most patients die as a consequence of cytopenia-associated complications, in the presence or absence of AML transformation. However a significant number of patients die of causes unrelated to the disorder. Although the French-American-British (FAB) classification4 has been relatively effective in categorizing MDS patients, it has some limitations in the clinical setting. These limitations include the wide range of marrow blast percentages in patients classified as having refractory anemia (RA) with excess blasts (RAEB) or chronic myelomonocytic leukemia, the failure to consider some important biologic determinants such as marrow cytogenetics, and the degree and number of cytopenias. These well-accepted problems in categorizing patients with MDS have led to the development of many additional risk-based stratification systems, which are reviewed critically in this chapter.

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