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Revised International Prognostic Scoring System for Myelodysplastic Syndromes
Abstract
The International Prognostic Scoring Sytem (IPSS) is an important standard for ssessing prognosis of primary untreated adult patients with myelodysplastic syndromes (MDS). To refine the IPSS, MDS patient databases from international institutions were coalesced to assemble a much larger combined database (Revised-IPSS [IPSS-R], n = 7012, IPSS, n = 816) for analysis. Multiple statistically weighted clinical features were used to generate a prognostic categorization model. Bone marrow cytogenetics, marrow blast percentage, and cytopenias remained the basis of the new system. Novel components of the current analysis included: 5 rather than 3 cytogenetic prognostic subgroups with specific and new classifications of a number of less common cytogenetic subsets, splitting the low marrow blast percentage value, and depth of cytopenias. This model defined 5 rather than the 4 major prognostic categories that are present in the IPSS. Patient age, performance status, serum ferritin, and lactate dehydrogenase were significant additive features for survival but not for acute myeloid leukemia transformation. This system comprehensively integrated the numerous known clinical features into a method analyzing MDS patient prognosis more precisely than the initial IPSS. As such, this IPSS-R should prove beneficial for predicting the clinical outcomes of untreated MDS patients and aiding design and analysis of clinical trials in this disease.
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“Among individuals who develop myelodysplastic syndrome (MDS), myeloproliferative neoplasm (MPN), or either disorder after CHIP, the rate of progression to acute myeloid leukemia (AML) varies significantly depending on the identity and characteristics of the pre-AML diagnosis.”
From Clonal haematopoiesis and dysregulation of the immune system (2023) · cites this paper as [219]
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“For example, over a one-year follow-up period, the rate of transformation to AML is 1-2% among patients with the MPN essential thrombocythemia 219 but more than 30% among patients with high-risk MDS 220 .”
From Clonal haematopoiesis and dysregulation of the immune system (2023) · cites this paper as [219]
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Cites
International scoring system for evaluating prognosis in myelodysplastic syndromes
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Cited by
Arber, D. A., Orazi, A., Hasserjian, R., Thiele, J., Borowitz, M. J., Le Beau, M. M., Bloomfield, C. D., Cazzola, M., & Vardiman, J. W. (2016) · Blood · doi:10.1182/blood-2016-03-643544 · Cited by 10,119
Self-citationKhoury, J. D., Solary, E., Abla, O., Akkari, Y., Alaggio, R., Apperley, J. F., Bejar, R., Berti, E., Busque, L., Chan, J. K. C., et al. (2022) · Leukemia · doi:10.1038/s41375-022-01613-1 · Cited by 4,274
Self-citationArber, D. A., Orazi, A., Hasserjian, R. P., Borowitz, M. J., Calvo, K. R., Kvasnicka, H.-M., Wang, S. A., Bagg, A., Barbui, T., Branford, S., et al. (2022) · Blood · doi:10.1182/blood.2022015850 · Cited by 2,945
Self-citationClinical and biological implications of driver mutations in myelodysplastic syndromes
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Self-citationLandscape of genetic lesions in 944 patients with myelodysplastic syndromes
Haferlach, T., Nagata, Y., Grossmann, V, Okuno, Y., Bacher, U., Nagae, G., Schnittger, S., Sanada, M., Kon, A., Alpermann, T., et al. (2014) · Leukemia · doi:10.1038/leu.2013.336 · Cited by 1,532
Metadata
- Authors
- Greenberg, P. L., Tuechler, H., Schanz, J., Sanz, G., Garcia-Manero, G., Solé, F., Bennett, J. M., Bowen, D., Fenaux, P., Dreyfus, F., Kantarjian, H., Kuendgen, A., Levis, A., Malcovati, L., Cazzola, M., Cermak, J., Fonatsch, C., Le Beau, M. M., Slovak, M. L., Krieger, O., Luebbert, M., Maciejewski, J., Magalhaes, S. M. M., Miyazaki, Y., Pfeilstöcker, M., Sekeres, M., Sperr, W. R., Stauder, R., Tauro, S., Valent, P., Vallespi, T., van de Loosdrecht, A. A., Germing, U., & Haase, D.
- Affiliations
- Stanford University Cancer Center, Stanford, CA;
- Hanusch Hospital, Boltzmann Institute for Leukemia Research, Vienna, Austria;
- Georg August Universität, Göttingen, Germany;
- Hospital Universitario La Fe, Valencia, Spain;
- The University of Texas, MD Anderson Cancer Center, Houston, TX;
- Hospital del Mar, Barcelona, Spain;
- James P. Wilmont Cancer Center, University of Rochester Medical Center, Rochester, NY;
- St James's University Hospital, Leeds, United Kingdom;
- Hôpital Avicenne, Assistance Publique–Hôpitaux de Paris (AP-HP)/University Paris XIII, Bobigny, France;
- Hôpital Cochin, AP-HP University of Paris V, Paris, France;
- Heinrich-Heine University Hospital, Düsseldorf, Germany;
- Antonio e Biagio e C Arrigo Hospital, Alessandria, Italy;
- Fondazione Istituti di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo and University of Pavia, Pavia, Italy;
- Institute of Hematology and Blood Transfusion, Praha, Czech Republic;
- Medical University of Vienna, Vienna, Austria;
- University of Chicago Comprehensive Cancer Research Center, Chicago, IL;
- Quest Diagnostics Nichols Institute, Chantilly, VA;
- Elisabethinen Hospital, Linz, Austria;
- University of Freiburg Medical Center, Freiburg, Germany;
- Cleveland Clinic, Cleveland, OH;
- Federal University of Ceara, Fortaleza, Brazil;
- Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan;
- University Hospital of Innsbruck, Innsbruck, Austria;
- University of Dundee, Scotland, United Kingdom;
- Hospital Universitario Vall d'Hebron, Barcelona, Spain; and
- VU University Medical Center, Amsterdam, The Netherlands
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 2012-09-20
- Volume
- 120
- Issue
- 12
- Pages
- 2454-2465
- ISSN
- 0006-4971
- 1528-0020
- Cited by
- 2,844
- References
- 3
- Language
- en
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- Greenberg, P. L., Tuechler, H., Schanz, J., Sanz, G., Garcia-Manero, G., Solé, F., Bennett, J. M., Bowen, D., Fenaux, P., Dreyfus, F., Kantarjian, H., Kuendgen, A., Levis, A., Malcovati, L., Cazzola, M., Cermak, J., Fonatsch, C., Le Beau, M. M., Slovak, M. L., . . . Haase, D. (2012). Revised International Prognostic Scoring System for Myelodysplastic Syndromes. Blood. https://doi.org/10.1182/blood-2012-03-420489