Article

Time-dependent prognostic scoring system for predicting survival and leukemic evolution in myelodysplastic syndromes

Department of Hematology, University of Pavia, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, 27100 Pavia, Italy.
Journal of Clinical Oncology (Impact Factor: 18.43). 08/2007; 25(23):3503-10. DOI: 10.1200/JCO.2006.08.5696
Source: PubMed

ABSTRACT The aims of this study were to identify the most significant prognostic factors in myelodysplastic syndromes (MDS) taking into account both their values at clinical onset and their changes in time and to develop a dynamic model for predicting survival and leukemic evolution that can be applied at any time during the course of the disease.
We studied a learning cohort of 426 MDS patients diagnosed at the Department of Hematology, San Matteo Hospital, Pavia, Italy, between 1992 and 2004, and a validation cohort of 739 patients diagnosed at the Heinrich-Heine-University Hospital, Düsseldorf, Germany, between 1982 and 2003. All patients were reclassified according to WHO criteria. Univariable and multivariable analyses were performed using Cox models with time-dependent covariates.
The most important variables for the prognostic model were WHO subgroups, karyotype, and transfusion requirement. We defined a WHO classification-based prognostic scoring system (WPSS) that was able to classify patients into five risk groups showing different survivals (median survival from 12 to 103 months) and probabilities of leukemic evolution (P < .001). WPSS was shown to predict survival and leukemia progression at any time during follow-up (P < .001), and its prognostic value was confirmed in the validation cohort.
WPSS is a dynamic prognostic scoring system that provides an accurate prediction of survival and risk of leukemic evolution in MDS patients at any time during the course of their disease. This time-dependent system seems particularly useful in lower risk patients and may be used for implementing risk-adapted treatment strategies.

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    • "Patients' performance status and fibrosis were graded by the Eastern Cooperative Oncology Group (ECOG) and European Myelofibrosis Network (EUMNET) [29] scores, respectively. Transfusion dependency was defined according to the WPSS [13] definition. "
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    ABSTRACT: Myelodysplastic syndromes (MDS) comprise bone marrow failure diseases with a diverse clinical outcome. For improved risk stratification, the International Prognostic Scoring System (IPSS) has recently been revised (IPSS-R). This single-centre study aimed to validate the IPSS-R and to evaluate prior prognostic scoring systems for MDS. We retrospectively analysed 363 patients diagnosed with MDS according to the FAB criteria between 2000 and 2012. The IPSS, MD Anderson Risk Model Score (MDAS), World Health Organisation (WHO)-classification based Prognostic Scoring System (WPSS), refined WPSS (WPSS-R), IPSS-R and MDS-Comorbidity Index (MDS-CI) were applied to 222 patients considered with primary MDS following the WHO criteria and their prognostic power was investigated. According to the IPSS-R, 18 (8%), 81 (37%), 50 (23%), 43 (19%) and 30 (13%) patients were classified as very low, low, intermediate, high and very high risk with, respectively, a median overall survival of 96 (95% Confidence interval (CI) not reached), 49 (95% CI 34-64), 22 (95% CI 0-49), 19 (95% CI 11-27) and 10 (95% CI 6-13) months (p<.000). The IPSS-R showed improved prognostic power as compared to the IPSS, MDAS, WPSS and WPSS-R. Furthermore, the MDS-CI refined the risk stratification of MDS patients stratified according to the IPSS-R. In conclusion, accounting for the disease status by means of the IPSS-R and comorbidity through the MDS-CI considerably improves the prognostic assessment in MDS patients. Copyright © 2014 Elsevier Ltd. All rights reserved.
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    • "Recently, the revised IPSS (IPSS-R) [4] for MDS incorporates a new classification of cytogenetic abnormalities [5], severity of peripheral cytopenias and different cut-off percentages for BM blasts, allowing identification of five, instead of the former four risk categories. In addition, transfusion dependence (TD; defined as having at least one red blood cell transfusion every 8 weeks over a period of 4 months) and degree of anemia were incorporated by the WHO-based Prognosis Scoring System (WPSS) providing risk stratification at any time during the course of the disease [6] [7]. However, the concept of LR-MDS, defined as low and intermediate- 1 risk categories by the IPSS has several limitations. "
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    • "Hematological malignancies such as leukemias, lymphomas, and multiple myelomas are common, accounting for about ∼9% of all newly diagnosed cancers (Sant et al 2010, Rodriguez-Abreu et al 2007) and most of them have a poor prognosis (Greenberg et al 1997, Malcovati et al 2007). In children and adolescents younger than twenty years, hematological malignancies are the most common type of cancer, responsible for more than 30% of all cancers (Greenberg et al 1997, Malcovati et al 2007). Most of these cancers originate in the bone marrow since this tissue is the main site of blood cell formation. "
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