Prognostic Impact of IPSS-R and Chromosomal Translocations in 751 Korean Patients with Primary Myelodysplastic Syndrome

Abstract

Chromosomal translocations are rare in myelodysplastic syndrome (MDS) and their impact on overall survival (OS) and response to hypomethylating agents (HMA) is unknown. The prognostic impact of the revised International Prognostic Scoring System (IPSS-R) and for chromosomal translocations was assessed in 751 patients from the Korea MDS Registry. IPSS-R effectively discriminated patients according to leukaemia evolution risk and OS. We identified 40 patients (5.3%) carrying translocations, 30 (75%) of whom also fulfilled complex karyotype criteria. Translocation presence was associated with a shorter OS (median, 12.0 versus 79.7 months, P < 0.01). Multivariate analysis demonstrated that translocations (hazard ratio [HR] 1.64 [1.06-2.63]; P = 0.03) as well as age, sex, IPSS-R, and CK were independent predictors of OS. In the IPSS-R high and very high risk subgroup (n = 260), translocations remained independently associated with OS (HR 1.68 [1.06-2.69], P = 0.03) whereas HMA treatment was not associated with improved survival (median OS, 20.9 versus 21.2 months, P = 0.43). However, translocation carriers exhibited enhanced survival following HMA treatment (median 2.1 versus 12.4 months, P = 0.03). Our data suggest that chromosomal translocation is an independent predictor of adverse outcome and has an additional prognostic value in discriminating patients with MDS having higher risk IPSS-R who could benefit from HMA treatment.

Fig 1

Kaplan-Meier survival curves of overall survival (A and B) and leukemia-free survival (C and D) in 751 patients with primary MDS stratified by IPSS and IPSS-R.

Fig 2

Overall survival according to presence of chromosomal translocation in (A) the whole population, and (B) patients with complex karyotype (CK), and (C) patients with IPSS-R poor and very poor cytogenetics.

Fig 3. Impact of hypomethylating agents (HMAs) on overall survival (OS) in IPSS-R high and very high risk group.

(A) Survival curves in patients treated with HMAs or not treated with HMAs. Impact of HMAs on OS in the subgroup of patients with chromosomal translocation (B) and without chromosomal translocation (C).