Validation of the molecular international prognostic scoring system in patients with myelodysplastic syndromes defined by international consensus classification
Wan‐Hsuan Lee, Ming-Tao Tsai, Cheng‐Hong Tsai, Feng‐Ming Tien, Min‐Yen Lo, Mei‐Hsuan Tseng, Yuan‐Yeh Kuo, Ming-Chih Liu, Yi‐Tsung Yang, Jui‐Che Chen, Jih‐Luh Tang, Hsun‐I Sun, Yi‐Kuang Chuang, Liang‐In Lin, Wen‐Chien Chou, Chien‐Chin Lin, Hsin‐An Hou, Hwei‐Fang Tien
Abstract
Myelodysplastic syndromes (MDS) have varied prognoses and require a risk-adapted treatment strategy for treatment optimization. Recently, a molecular prognostic model (Molecular International Prognostic Scoring System [IPSS-M]) that combines clinical parameters, cytogenetic abnormalities, and mutation topography was proposed. This study validated the IPSS-M in 649 patients with primary MDS (based on the 2022 International Consensus Classification [ICC]) and compared its prognostic power to those of the IPSS and revised IPSS (IPSS-R). Overall, 42.5% of the patients were reclassified and 29.3% were up-staged from the IPSS-R. After the reclassification, 16.9% of the patients may receive different treatment strategies. The IPSS-M had greater discriminative potential than the IPSS-R and IPSS. Patients with high, or very high-risk IPSS-M might benefit from allogeneic hematopoietic stem cell transplantation. IPSS-M, age, ferritin level, and the 2022 ICC categorization predicted outcomes independently. After analyzing demographic and genetic features, complementary genetic analyses, including KMT2A-PTD, were suggested for accurate IPSS-M categorization of patients with ASXL1, TET2, STAG2, RUNX1, SF3B1, SRSF2, DNMT3A, U2AF1, and BCOR mutations and those classified as MDS, not otherwise specified with single lineage dysplasia/multi-lineage dysplasia based on the 2022 ICC. This study confirmed that the IPSS-M can better risk-stratified MDS patients for optimized therapeutic decision-making.