Validation of the new comprehensive cytogenetic scoring system (NCCSS) on 630 consecutive de novo MDS patients from a single institution

Abstract

This study evaluated whether the NCCSS truly improves the prognostic stratification of 630 consecutive de novo MDS patients and established which cytogenetic grouping [NCCSS or International Prognostic Scoring System (IPSS)], when combined with the WHO classification, best predicted the clinical outcome of myelodysplastic syndromes (MDS). The frequency of chromosomal defects was 53.8%. Clinical parameters, including number of cytopenias, WHO classification, IPSS cytogenetic categories and scores, NCCSS were all relevant for overall survival (OS) and leukemia-free survival (LFS) and were included in six distinct multivariate models compared by the Akaike Information Criterion (AIC). The most effective model to predict OS included the number of cytopenias, the WHO classification and the NCCSS, whereas the model including the number of cytopenias, blast cell percentage and the NCCSS and the model including the number of cytopenias the WHO classification and the NCCSS were almost equally effective to predict LFS. In conclusion, the NCCS (i) improves the prognostic stratification of the good and poor IPSS cytogenetic categories by introducing the very good and the very poor categories; (ii) is still incomplete in establishing the prognostic relevance of rare/double defects, (ii) applied to patients who receive supportive treatment only identifies five different prognostic subgroups, but applied to patients treated with specific therapies reveals only a trend toward a significantly different OS and LFS when patients of the poor and intermediate cytogenetic categories are compared, (iii) combined with the WHO classification is much more effective than the IPSS in predicting MDS clinical outcome. Am. J. Hematol. 88:120-129,

Evaluation of revised IPSS cytogenetic risk stratification and prognostic impact of monosomal karyotype in 783 patients with primary myelodysplastic syndromes

“…We cannot explain why our findings are different from those of other investigators [8,13] but we show that our patient cohort behaved as expected when the original IPSS cytogenetic risk stratification was applied [4]. More importantly, we demonstrate the additional value of considering MK as a separate prognostic category, and our observations in this regard are consistent with the adverse prognostic impact of MK in other myeloid malignancies including acute myeloid leukemia and primary myelofibrosis [9,14].…”

Myelodysplastic syndromes in South America: A multinational study of 1080 patients

“…3). The median age at diagnosis seems to vary in different countries, being lower in "Eastern" than in "Western" countries: 59 years old in China [22], 60 in Japan [24], 58 in Korea [25], 56 in Thailand [26], and 65 in Taiwan [27], 77 in US [28], 72-73 in Germany [15,16,29], and 65-71 in Italy [15,16,30,31]. However, a new report using a population-based registry data showed a median age of 76 years for Japan [32].…”

Cytogenetic clonal evolution in myelodysplastic syndromes is associated with inferior prognosis

“…Complex karyotypes were divided into “poor” when there were 3 abnormalities and “very poor” when there were >3 abnormalities. The IPSS and IPSS‐R have been validated repeatedly . However, to the best of our knowledge, the prognostic impact of chromosomal aberrations has been evaluated only based on their presence at the time of diagnosis, whereas the significance of chromosomal changes developing during the disease has largely been neglected.…”