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. Author manuscript; available in PMC: 2013 Sep 10.
Published in final edited form as: Am J Med. 2013 Apr;126(4):e25. doi: 10.1016/j.amjmed.2012.08.013

Prognostication in Myelodysplastic Syndromes: Beyond the International Prognostic Scoring System (IPSS)

Amer M Zeidan a, B Douglas Smith a, Rami S Komrokji b, Steven D Gore a
PMCID: PMC3768129  NIHMSID: NIHMS492567  PMID: 23507216

To the Editor:

We read with interest the comprehensive review “Myelodysplastic Syndromes: Therapy and Outlook” by Lyons published in The American Journal of Medicine in July 2012.1 We agree with Lyons that the International Prognostic Scoring System (IPSS) is a valuable prognostic tool in myelodysplastic syndromes. Nonetheless, there is increasing evidence that a significant group of patients with IPSS low-risk myelodysplastic syndromes has a more aggressive disease with shorter survival than predicted. Identification of such patients would allow for increased surveillance and possibly impact their disease through earlier intervention with disease-modifying therapies.

The IPSS was originally derived from a cohort of untreated patients at time of diagnosis, and it did not account for multilineage dysplasia, transfusion-dependence, or severity of cytopenias.2 To overcome these limitations, the World Health Organization Prognostic Scoring System incorporated transfusion-dependence and multilineage dysplasia in a flexible scoring instrument to be used at different time points and further refine prognosis in myelodysplastic syndromes.3 The MD Anderson group developed a new prognostic model (MDAS) by adding age, performance status, and degree of thrombocytopenia.4 The model was externally validated where 25% of IPSS low-risk myelodysplastic syndromes patients were up-staged by the MDAS.5 Four subgroups among the IPSS low-risk myelodysplastic syndromes patients were identified with significantly different median overall survival (OS) of 93, 53, 31, and 18 months.5 A specific low-risk MDAS model also was proposed.6 Patients were stratified into 3 categories using the sum of points generated from cytogenetics, hemoglobin level, platelet count, bone marrow blast percentage, and age. Bejar et al7 validated the low-risk MDAS model in an independent cohort of patients, and separated these patients into 3 risk categories with significantly different OS.

Recognizing these shortcomings, a revised IPSS (IPSS-R) was recently proposed.8 The IPSS-R used 5 rather than 3 cytogenetic prognostic subgroups, split the low bone marrow blast percentage, and used the depth of cytopenias to generate 5 prognostic categories. While this system still needs further confirmation, it has become clear that efforts to better refine prognosis beyond the IPSS are critically important to help clinical decision-making. It also is expected that additional molecular, epigenetic, and immunologic determinants will contribute to improving our prognostic tools and, more importantly, allow tailoring therapy accordingly.

Acknowledgments

Funding: None.

Footnotes

Conflict of Interest: None.

Authorship: All authors had access to the data and played a role in writing this manuscript.

References

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