Prognostic models in myelodysplastic syndromes

Myelodysplastic syndromes are extremely heterogeneous disorders, which range from indolent disease with near-normal life expectancy for patients, to forms similar to acute myeloid leukaemia. This clinical heterogeneity possibly results from the different somatic mutations that caused the original clonal proliferation of myelodysplastic stem cells. Treatment that is linked to risk is essential for practical disease management in conditions with such a highly variable clinical course. Prognostic scoring systems that combine various clinical and haematological features are used to define risk associated with myelodysplastic syndromes. 1 Malcovati L Hellström-Lindberg E Bowen D et al. Diagnosis and treatment of primary myelodysplastic syndromes in adults: recommendations from the European LeukemiaNet. Blood. 2013; 122: 2943-2964 Crossref PubMed Scopus (519) Google Scholar The International Prognostic Scoring System (IPSS) 2 Greenberg P Cox C LeBeau MM et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood. 1997; 89: 2079-2088 Crossref PubMed Google Scholar was developed in 1997 and uses factors such as bone marrow blasts, cytogenetic abnormalities, and number of cytopenias to predict patient prognoses. The IPSS has become the benchmark for clinical decision-making, clinical trials, and drug approval. Despite its usefulness and reproducibility, the IPSS has limitations, including the fact that low and intermediate-1 risk scores are unable to identify a homogeneous clinical outcome for patients with such scores, as some patients scored within these groups still have a poor outcome. 2 Greenberg P Cox C LeBeau MM et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood. 1997; 89: 2079-2088 Crossref PubMed Google Scholar Use of newer prognostic indices for patients with myelodysplastic syndromes in the low and intermediate-1 risk categories: a population-based studyRecently proposed prognostic indices for myelodysplastic syndromes can be used to improve identification of patients with poor prognoses in the group of patients with intermediate-1 IPSS scores, who could potentially benefit from a high-risk treatment approach. Full-Text PDF