Risk factors and their relationship to prognosis in myelodysplastic syndromes

Recent efforts have been directed at improving the methodology for predicting clinical outcomes in patients with myelodysplastic syndromes (MDS). This review focuses on the development of a consensual, prognostic, risk-based analysis system generated by the International MDS Risk Analysis Workshop. In the workshop, cytogenetic, morphological, and clinical data were combined and collated from a relatively large group of patients with primary MDS. Critical prognostic variables were evaluated using the data set. Based on these findings, the International Prognostic Scoring System (IPSS) was developed, compared with other systems, and shown to provide more accurate prognoses regarding survival and evolution to acute myeloid leukemia in MDS patients. The improvement was due to several features of the workshop model: more refined cytogenetic categorization, inclusion of cytopenias, improved subdivision of marrow blast percentages, four subgroups defining outcome, and separate stratification for age. The IPSS should result in better-defined clinical outcomes in MDS and provide a framework for future studies determining the possible role of molecular determinants (e.g. oncogenes, tumor suppressor genes, cytokine expression and responsiveness) for evaluating prognoses. The IPSS will likely prove useful in the design and analysis of therapeutic trials in MDS as well as in patient management.