Revised International Prognostic Scoring System for myelodysplasia

Prognosis of myelodysplastic syndromes (MDS)

1. Sum the scores for each feature

ScoreCyto­genetic cate­gory*Marrow blasts (%)Hemo­globin (g/dL)Plate­lets (x 109/L)Neutro­phils (x 109/L)
0Very good≤ 2≥ 10≥ 100≥ 0.8
0.5---50 - 99< 0.8
1.0Good2.1 - 4.98 - 9.9< 50-
1.5--< 8--
2.0Inter­mediate5 - 10--
3.0Poor> 10---
4.0Very poor----
*Cytogenetic categories:

  • Very good: -Y, del(11q)
  • Good: normal, del(5q), del(12p), del(20q), double including del(5q)
  • Intermediate: del(7q), +8, +19, i(17q), any other single or double independent clones
  • Poor: −7, inv(3)/t(3q)/del(3q), double including −7/del(7q), complex with 3 abnormalities
  • Very poor: complex with >3 abnormalities

2. Use summed score to determine risk category and prognosis

ScoreRisk categoryMedian OS
(years)
Median time to 25% AML risk (years)
≤ 1.5Very low8.8NR
2.0 - 3.0Low5.310.8
3.5 - 4.5Intermediate3.03.2
5.0 - 6.0High1.61.4
> 6.0Very high0.80.73

The Revised IPSS (IPSS-R) was based on multivariate analysis of survival and freedom from progression to acute myeloid leukaemia of 7012 patients with de novo untreated myelodysplastic syndromes.1 Weighted scores for each of five disease features are used to generate five prognostic categories. In addition to the five disease features used to calculate the IPSS-R, patient age, performance status, ferritin, and lactate dehydrogenase were significantly associated with survival, but not with acute myeloid leukemia transformation.

In comparison with the original IPSS, the IPSS-R has one additional risk category,  two additional cytogenetic risk categories (incorporating some lower-prevalence cytogenetic abnormalities),  splits the lower marrow blast percentages, and incorporates the degree of each individual cytopenia. The IPSS-R provides more precise prognostic values than the original IPSS.