IPSS‐R in 555 Taiwanese patients with primary MDS: Integration of monosomal karyotype can better risk‐stratify the patients

Abstract

The revised International Prognostic Scoring System (IPSS-R) was recently developed to better assess the clinical outcome of adult patients with myelodysplastic syndrome (MDS). In this study, we aimed to investigate the prognostic impact of this new risk model on 555 MDS patients in Taiwan. Generally, the IPSS-R could discriminate MDS patients regarding risk of leukemia evolution and overall survival in our cohort and it further refined prognostic stratification in all IPSS risk categories. However, we could not find the intergroup difference between IPSS-R very low and low risk subgroups in both leukemia-free survival (LFS) and overall survival (OS). IPSS-R couldn't distinguish the prognosis between very good and good and between good and intermediate risk cytogenetic categories in OS, and between very good and good and between intermediate and poor cytogenetic-risk categories in LFS, either. On the other hand, incorporation of monosomal karyotype (MK) into IPSS-R could further stratify MDS patients with higher-risk IPSS-R (intermediate, high and very high risk) into four groups, rather than three groups, with different OS (P < 0.001). Intriguingly, patients receiving allogeneic hematopoietic stem cell transplantation had longer survival than those without in the IPSS-R high and very high, but not other risk groups. Similarly, patients treated with hypomethylating agents had better survival than those not in the IPSS-R very high risk group. In conclusion, IPSS-R can risk-stratify MDS patients in Taiwan but with some limitations, especially in very low risk category, and MK has additional prognostic value in discriminating MDS patients with higher-risk IPSS-R.

Monosomal karyotypes apart from complex karyotypes independently predict the outcome of myelodysplastic syndrome patients using a fluorescence in situ hybridization panel and conventional cytogenetics

“…Koenecke et al reported that MK is a negative predictor of outcome in the poor risk group (but not the very poor risk group) of the IPSS‐R classification in MDS patients after HSCT. In publications on Asian populations, MKs had an independent adverse prognostic value . For example, a Korean report noted that MKs may predict poor survival but for non‐CK status only .…”

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].…”

Impact of the revised International Prognostic Scoring System, cytogenetics and monosomal karyotype on outcome after allogeneic stem cell transplantation for myelodysplastic syndromes and secondary acute myeloid leukemia evolving from myelodysplastic syndromes: a retrospective multicenter study of the European Society of Blood and Marrow Transplantation

“…32 The predictive value of cytogenetic information for outcome after allogeneic SCT has been reported by others, not only for the 3-group-IPSSclassification, 33 but also for the 5-group IPSS-R-classification. [13][14][15] In all studies, unfavorable cytogenetic information, as classified according to the IPSS or IPSS-R classification, predicted worse patient outcome. However, this study is the first to evaluate formally the added value of the 5-group IPSS-R classification compared to the 3-group classification in the setting of SCT for predictive performance and to show that the relevant distinction in categories in this context is standard-poor-very poor when other risk factors are taken into account.…”

“…number of marrow blasts, time from diagnosis to transplant, prior chemotherapeutic treatment and remission status), the karyotype of the disease seems to be most predictive for relapse-free survival (RFS) after transplantation. [5][6][7][8][9][10][11][12][13][14][15] However, most studies used the International Prognostic Scoring System (IPSS) cytogenetic risk score (good, intermediate, and poor cytogenetic risk groups) for the analysis of relapse and OS after SCT in MDS patients. 16 In 2012, a new 5-group cytogenetic scoring system with a refined cytogenetic risk prediction in MDS patients not undergoing SCT was proposed.…”