What are the risk categorizations and predicted clinical outcomes of the International Prognostic Scoring System (IPSS) score for myelodysplastic syndrome (MDS)?

Updated: Jul 24, 2018
  • Author: Emmanuel C Besa, MD; Chief Editor: Koyamangalath Krishnan, MD, FRCP, FACP  more...
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Answer

Calculation of the IPSS-R score in 7012 patients with MDS resulted in the following risk categorizations [18] :

  • Very low: 19%
  • Low: 38%
  • Intermediate: 20%
  • High: 13%
  • Very high: 10%

Clinical outcome according to IPSS-R risk category in those patients is outlined in Table 5, below.

Table 5. Clinical outcome by IPSS-R risk category (Open Table in a new window)

 

IPSS-R Risk Category

 

Very Low

Low

Intermediate

High

Very High

Clinical Outcome

Median survival (years)

8.8

5.3

3.0

1.6

0.8

Median time to 25% acute myelogenous leukemia evolution (years)

NR

10.8

3.2

1.4

0.7

Classification of the subtypes or categories of MDS has changed from the FAB classification to the WHO classification. (See Table 6, below.)

Table 6. Categories of FAB classification versus WHO classification for myelodysplastic syndrome (MDS) (Open Table in a new window)

FAB

Classification

WHO–2004

Classification

WHO–2008

Classification

RA

RA RCMD 5q-

RCUD RCMD 5q-

RARS

RARS RCMD-RS

RARS RCMD-RS RARS-T

RAEB

RAEB-1 RAEB-2

RAEB-1 RAEB-2

CMML

CMML-1 CMML-2

CMML-1 CMML-2

RAEB-T

AML

AML

FAB – French-American-British Cooperative Group; WHO – World Health Organization; RA – Refractory anemia; RARS – RA with ringed sideroblasts; RAEB – RA with excess blasts; RAEB-T – RAEB in transition to AML; AML – Acute myelogenous leukemia; CMML – Chronic myelomonocytic leukemia; RCMD – Refractory cytopenia with multilineage dysplasia; RCUD – Refractory cytopenia with unilineage dysplasia


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