International scoring system for evaluating prognosis in myelodysplastic syndromes

Greenberg, Peter; Cox, Christopher; LeBeau, Michelle M.; Fenaux, Pierre; Morel, Pierre; Sanz, Guillermo; Sanz, Miguel; Vallespi, Teresa; Hamblin, Terry; Oscier, David; Ohyashiki, Kazuma; Toyama, Keisuke; Aul, Carlo; Mufti, Ghulam; Bennett, John

doi:10.1182/blood.v89.6.2079

Cited by 4,427Acute Myeloid Leukemia ResearchRetinoids in leukemia and cellular processesHematopoietic Stem Cell Transplantation

International scoring system for evaluating prognosis in myelodysplastic syndromes

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Greenberg, Peter, Cox, Christopher, LeBeau, Michelle M., Fenaux, Pierre, Morel, Pierre, Sanz, Guillermo, Sanz, Miguel, Vallespi, Teresa +7 more·1997·Blood

DOI 10.1182/blood.v89.6.2079

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Abstract

Despite multiple disparate prognostic risk analysis systems for evaluating clinical outcome for patients with myelodysplastic syndrome (MDS), imprecision persists with such analyses. To attempt to improve on these systems, an International MDS Risk Analysis Workshop combined cytogenetic, morphological, and clinical data from seven large previously reported risk-based studies that had generated prognostic systems. A global analysis was performed on these patients, and critical prognostic variables were re-evaluated to generate a consensus prognostic system, particularly using a more refined bone marrow (BM) cytogenetic classification. Univariate analysis indicated that the major variables having an impact on disease outcome for evolution to acute myeloid leukemia were cytogenetic abnormalities, percentage of BM myeloblasts, and number of cytopenias; for survival, in addition to the above, variables also included age and gender. Cytogenetic subgroups of outcome were as follows: “good” outcomes were normal, −Y alone, del(5q) alone, del(20q) alone; “poor” outcomes were complex (ie, ≥3 abnormalities) or chromosome 7 anomalies; and “intermediate” outcomes were other abnormalities. Multivariate analysis combined these cytogenetic subgroups with percentage of BM blasts and number of cytopenias to generate a prognostic model. Weighting these variables by their statistical power separated patients into distinctive subgroups of risk for 25% of patients to undergo evolution to acute myeloid leukemia, with: low (31% of patients), 9.4 years; intermediate-1 (INT-1; 39%), 3.3 years; INT-2 (22%), 1.1 years; and high (8%), 0.2 year. These features also separated patients into similar distinctive risk groups for median survival: low, 5.7 years; INT-1, 3.5 years; INT-2, 1.2 years; and high, 0.4 year. Stratification for age further improved analysis of survival. Compared with prior risk-based classifications, this International Prognostic Scoring System provides an improved method for evaluating prognosis in MDS. This classification system should prove useful for more precise design and analysis of therapeutic trials in this disease.

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Despite advances in understanding the genetic basis of MDS and AML, cure rates have not substantially improved in the last 40 years.
“Despite tremendous advances in understanding the genetic basis of MDS and AML, cure rates have not substantially improved in the last 40 years (45).”
From Txnip Enhances Fitness of Dnmt3a -Mutant Hematopoietic Stem Cells via p21 (2022) · cites this paper as [45]
The paper’s prognostic-classification section reports survival and progression to AML, and its leukemia-free-survival section discusses outcomes by risk group; page numbers are unavailable in our copy. Neither addresses whether MDS and AML cure rates changed over 40 years despite advances in genetic understanding (searched: cure rates, treatment progress, and decades). (LLM, conf=0.76)
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Self-citation
Clinical Prognostic Factors for Survival and Risk of Progression to Acute Myeloid Leukemia in Patients With Myelodysplastic Syndromes With < 10% Marrow Blasts and Non-Unfavorable Cytogenetic Categories
Falantes, J. F., Calderón, C., Márquez-Malaver, F. J., Alonso, D., Noya, A. M., Carrillo, E., Martino, M. L., Montero, I., González, J., Parody, R., et al. (2012) · Clinical Lymphoma Myeloma & Leukemia · doi:10.1016/j.clml.2012.09.013 · Cited by 19 · see citing works
New Comprehensive Cytogenetic Scoring System for Primary Myelodysplastic Syndromes (MDS) and Oligoblastic Acute Myeloid Leukemia After MDS Derived From an International Database Merge
Schanz, J., Tüchler, H., Solé, F. G., Mallo, M., Luño, E., Cervera, J., Granada, I., Hildebrandt, B., Slovak, M. L., Ohyashiki, K., et al. (2012) · Journal of Clinical Oncology · doi:10.1200/jco.2011.35.6394 · Cited by 688 · see citing works
Self-citation
Growing Evidence for an Underestimation of Poor-Risk Cytogenetics in the International Prognostic Scoring System in Myelodysplastic Syndromes
Steidl, C., Schanz, J., Pfeilstöcker, M., Nösslinger, T., Hildebrandt, B., Kuendgen, A., Lübbert, M., Kunzmann, R., Giagounidis, A. A. N., Aul, C., et al. (2007) · Clinical Leukemia · doi:10.3816/clk.2007.n.029 · Cited by 8 · see citing works
Self-citation
Coalesced Multicentric Analysis of 2,351 Patients With Myelodysplastic Syndromes Indicates an Underestimation of Poor-Risk Cytogenetics of Myelodysplastic Syndromes in the International Prognostic Scoring System
Schanz, J., Steidl, C., Fonatsch, C., Pfeilstöcker, M., Nösslinger, T., Tuechler, H., Valent, P., Hildebrandt, B., Giagounidis, A. A. N., Aul, C., et al. (2011) · Journal of Clinical Oncology · doi:10.1200/jco.2010.28.3978 · Cited by 159 · see citing works
Self-citation

Authors: Greenberg, P., Cox, C., LeBeau, M. M., Fenaux, P., Morel, P., Sanz, G., Sanz, M., Vallespi, T., Hamblin, T., Oscier, D., Ohyashiki, K., Toyama, K., Aul, C., Mufti, G., & Bennett, J.
Affiliations: Tokyo Medical University (JP)
From the Hematology Divisions of Stanford University Medical Center, Stanford, CA; the Rochester Medical Center, Rochester, NY; the University of Chicago, Chicago, IL; Centre Hospitalier Universitaire, Lille, France; Hospital La Fe, Valencia, Spain; Hospital Vall d'Hebron, Barcelona, Spain; the Royal Bournemouth Hospital, Bournemouth, UK; the Tokyo Medical College, Tokyo, Japan; the Heinrich Heine University, Dusseldorf, Germany; and the King's College Hospital, London, UK.
ORCID: Greenberg, Peter
Cox, Christopher
Fenaux, Pierre
Morel, Pierre
Sanz, Guillermo
Sanz, Miguel
Oscier, David
Ohyashiki, Kazuma
Toyama, Keisuke
Mufti, Ghulam
Bennett, John
Venue: Blood
Publisher: American Society of Hematology
Published: 1997-03-15
Volume: 89
Issue: 6
Pages: 2079-2088
ISSN: 0006-4971 (ISSN-L)
1528-0020
DOI: 10.1182/blood.v89.6.2079
OpenAlex: W187238933
Cited by: 4,427
References: 33
Citations by year: 2026: 45 · 2025: 69 · 2024: 93 · 2023: 124 · 2022: 131 · 2021: 155 · 2020: 167 · 2019: 144 · 2018: 174 · 2017: 226 · 2016: 183 · 2015: 205 · 2014: 232 · 2013: 253 · 2012: 256
FWCI: 36.61
Type: journal article
Language: en
Open access: bronze
doi.org
Locations: Blood (publishedVersion)
PubMed (publishedVersion)
Topics: Health Sciences › Medicine › Hematology › Acute Myeloid Leukemia Research
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Retinoids in leukemia and cellular processes
Health Sciences › Medicine › Hematology › Hematopoietic Stem Cell Transplantation
Keywords: myelodysplastic syndrome, prognostic score, International Prognostic Scoring System, risk stratification, bone marrow cytogenetics, acute myeloid leukemia transformation, bone marrow blasts, cytopenia, survival, del(5q), del(20q)
MeSH terms: Acute Disease, Adolescent, Adult, Age Factors, Aged, Bone Marrow, Female, Follow-Up Studies, Humans, Leukemia, Myeloid, Male, Middle Aged, Myelodysplastic Syndromes, Prognosis, Sex Factors, Multivariate Analysis, Survival Analysis, Risk Assessment

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