Visualizing the Effect of
IDH Mutations in MDS

Visualizing the Effect of IDH Mutations in MDS

Using the IPSS-M Risk Calculator

Evolution of the International
Prognostic Scoring System (IPSS)

The IPSS was developed in 1997 and served as a standard for assessing the risks of myelodysplastic syndromes (MDS).1 Patients were assigned to one of four main categories: low, intermediate-1, intermediate-2, or high risk.1 In 2012, the system was updated to the IPSS-R (revised), which expanded risk stratification into five categories (very low, low, intermediate, high, very high).1,2 These systems, however, were limited to hematologic parameters and cytogenetic abnormalities.2 They did not consider somatic mutations in risk stratification.2

The IPSS was developed in 1997 and served as a standard for assessing the risks of myelodysplastic syndromes (MDS).1 Patients were assigned to one of four main categories: low, intermediate-1, intermediate-2, or high risk.1 In 2012, the system was updated to the IPSS-R (revised), which expanded risk stratification into five categories (very low, low, intermediate, high, very high).1,2 These systems, however, were limited to hematologic parameters and cytogenetic abnormalities.2 They did not consider somatic mutations in risk stratification.2

Evolving risk classification1,2

In 2022, the IPSS-M (Molecular) system was introduced, and unlike its predecessor, incorporated gene mutations to provide more comprehensive and meaningful prognostic information.1,2

Evolving risk classification1,2

In 2022, the IPSS-M (Molecular) system was introduced, and unlike its predecessor, incorporated gene mutations to provide more comprehensive and meaningful prognostic information.1,2

By evaluating the combination of hematologic parameters, cytogenetic abnormalities, and somatic mutations in 31 genes, the IPSS-M generates a unique risk score for individual patients.2 Furthermore, it has led to the derivation of six IPSS-M risk categories, unveiling distinct prognostic differences among patients.2 As a result, 46% of patients have been reclassified when compared to the previous IPSS-R.2 Overall, the IPSS-M offers improved prognostic accuracy across a greater range of patients, and is a valuable tool for evaluating the risk of MDS and progression to AML.2

By evaluating the combination of hematologic parameters, cytogenetic abnormalities, and somatic mutations in 31 genes, the IPSS-M generates a unique risk score for individual patients.2 Furthermore, it has led to the derivation of six IPSS-M risk categories, unveiling distinct prognostic differences among patients.2 As a result, 46% of patients have been reclassified when compared to the previous IPSS-R.2 Overall, the IPSS-M offers improved prognostic accuracy across a greater range of patients, and is a valuable tool for evaluating the risk of MDS and progression to AML.2

94% of patients with MDS have at least one oncogenic mutation. 46% of patients are restratified to a new risk category in the IPSS-M from their original IPSS-R category. 74% of restratified patients are upstaged into a higher risk category when mutations are considered.2

Key Points

  • The IPSS-M incorporates gene mutations to provide more comprehensive and meaningful prognostic information2
  • The IPSS-M changes led to a reclassification of 46% of patients2
  • The IPSS-M is capable of improved prognostic accuracy across a greater range of patients2
The IPSS-M incorporates gene mutations to provide more comprehensive and meaningful prognostic information2 The IPSS-M changes led to a reclassification of 46% of patients2 The IPSS-M is capable of improved prognostic accuracy across a greater range of patients2

IPSS-M Risk Categories and
Associated Clinical Outcomes2

Increased risk category decreases median leukemia-free survival (LFS) and median overall survival (OS) and increases the risk of AML transformation.2

Visualizing the Effect of
Mutations on MDS Patient Risk

Visualizing the Effect of Mutations on MDS Patient Risk

Using the IPSS-M Risk Calculator

The most recent version of the IPSS-M incorporates molecular gene mutations (such as IDH1 and IDH2) to improve risk stratification and therapeutic decision-making in MDS.2,3

The addition of molecular mutations to a baseline profile may “upstage” an MDS patient into a higher risk category.2–4

The most recent version of the IPSS-M incorporates molecular gene mutations (such as IDH1 and IDH2) to improve risk stratification and therapeutic decision-making in MDS.2,3

The addition of molecular mutations to a baseline profile may “upstage” an MDS patient into a higher risk category.2–4

Note that IPSS-M results are less confident with missing mutation data.3

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References:

1. Greenberg PL, Tuechler H, Schanz J, et al. Blood. 2012;120(12):2454-2465. doi:10.1182/blood-2012-03-420489 2. Bernard E, Tuechler H, Greenberg PL, et al. NEJM Evidence. 2022;1(7). doi:10.1056/EVIDoa2200008 3. IPSS-M Risk Calculator website. https://mds-risk-model.com/. Accessed March 25, 2023. 4. Cazzola M. Hematology. 2022;2022(1):375-381. doi:10.1182/hematology.2022000349 5. Aguirre LE, Al Ali N, Sallman DA, et al. Leukemia. Published online May 5, 2023. doi:10.1038/s41375-023-01910-3 6. Thol F, Weissinger EM, Krauter J, et al. Haematologica. 2010;95(10):1668-1674. doi:10.3324/haematol.2010.025494 7. Abbas S, Lugthart S, Kavelaars FG, et al. Blood. 2010;116(12):2122-2126. doi:10.1182/blood-2009-11-250878