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International scoring system for evaluating prognosis in myelodysplastic
syndromes
P Greenberg, C Cox, MM LeBeau, P Fenaux, P Morel, G Sanz, M Sanz, T Vallespi, T Hamblin, D Oscier, K Ohyashiki, K Toyama, C Aul, G Mufti and J Bennett
Hematology Division, Stanford University Medical Center, CA 94305, USA.
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, > or = 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.
Volume 89,
Issue 6,
pp. 2079-2088,
03/15/1997
Copyright © 1997 by The American Society of Hematology
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