How is risk stratified for myelodysplastic syndromes (MDS)?

Updated: Oct 18, 2019
  • Author: Matthew C Foster, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Answer

Although several prognostic scoring systems have been developed, [4, 5] the most widely used has been the IPSS. [2] The IPSS-R is a recdent revision of the IPSS that refines cytogenetic prognostic categorization and weights the prognostic score according to the severity of cytopenias in addition to the bone marrow blast percentage. The sum of the assigned points yields a prognostic score, which defines survival and risk of progression to AML. In addition to the clinical and pathologic variables included in the IPSS-R, point mutations in genes such as TP53, EZH2, ETV6, RUNX1, and ASXL1 have been shown to identify patients at risk for shortened survival or transformation to acute leukemia. [6] See the tables below.

Table 2. IPSS-R assignment of score (Open Table in a new window)

 

Score Value

Prognostic variable

0

0.5

1

1.5

2

3

4

Marrow blasts (%)

< 2

-

2 - < 5

-

5-10

>10

-

Karyotype

Very Good

-

Good

Intermediate

Poor

Very Poor

Hemoglobin (g/dL)

≥10

-

8 - < 10

< 8

-

-

-

Absolute Neutrophil Count (x 109/L)

≥0.8

< 0.8

-

-

-

-

-

Platelet Count (x 109/L)

≥100

50 - < 100

< 50

-

-

-

-

Very Good Karyotype: del(11q), -Y; Good karyotype: 46XX, 46XY, del(5q), del(20q); poor karyotype: complex (≥3 unrelated abnormalities) or chromosome 7 abnormalities; intermediate karyotype: karyotypes not defined as good or poor.

 

 

 

Table 3. IPSS-R risk groups, by sum of score [7] (Open Table in a new window)

Risk Category

(% IPSS-R population)

Overall Score

Median Survival (years)

Time for 25% of patients to progress to AML

Very Low (19)

≤1.5

8.8

Not reached

Low (38)

2-3

5.3

10.8

Intermediate (20)

3.5-4.5

3.0

3.2

High (13)

5-6

1.6

1.4

Very High (10)

>6

0.8

0.73

AML = acute myeloid leukemia; IPSS-R = Revised International Prognostic Scoring System.


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