Dr Komrokji on the Classification of Patients With Myelodysplastic Syndromes

Rami Komrokji, MD, vice chair, Malignant Hematology Department, head, the Leukemia and MDS Section, Moffitt Cancer Center, senior member, the Malignant Hematology and Experimental Therapeutics Program, professor in Medicine & Oncologic Sciences, the College of Medicine, the University of South Florida, discusses key findings from an expanded analysis of the classification of patients with myelodysplastic syndromes (MDS).

As knowledge of disease biology has improved over the past decade, the classification of MDS has significantly evolved. In 2022, the updated International Consensus Classification (ICC) andWorld Health Organization (WHO) classification systems introduced several changes to diagnostic criteria and prognostication of these neoplasms.

Understanding the differences and similarities between these guidelines could help improve the standardization of treatment approaches in this space. Accordingly, a study was designed to validate, compare, and harmonize the use of these classification systems in a large, international patient cohort. Two large, annotated datasets from the Moffitt Cancer Center and GenoMed4all, were analyzed in this trial. A total of 7017 patients with molecular data were included in the trial and reclassified according to WHO and ICC criteria.

Results from this analysis showed that patients with SF3B1 mutations had superior survival outcomes vs other genetically defined MDS subsets, Komrokji begins. This category encompasses 12% to 13% of all patients with MDS. Notably, there were minimal differences between WHO and ICC definitions for this subgroup, Komrokji adds.

Further analysis of patients categorized as having MDS with ring sideroblasts (MDS-RS) showed that MDS-RS SF3B1 wild-type disease was less common than MDS with low blasts (MDS-LB), despite the prevalence of the RS phenotype in those with SF3B1 mutations, Komrokji continues. Moreover, patients who were MDS-RS SF3B1 wild-type had similar outcomes to those with MDS-LB in both WHO and ICC cohorts, he says. Although the MDS-RS category may not be used in the future, the retention of ring sideroblasts could still have utility in countries that do not have the technology to directly assess SF3B1, Komrokji notes.

Additionally, deletion 5q was associated with favorable survival outcomes and is present in 5% of all MDS cases, Komrokji states. Conversely, patients with bi-allelic TP53 inactivation had the least favorable outcomes, and account for approximately 10% of all MDS cases, Komrokji concludes.