Canterbury DHB

Context

Assessment of Prognosis

There are a number of scoring systems available to assess the prognosis of patients with myelodysplasia. The IPSS was first published in 1997 (Greenberg et al, 1997) and until recently was the standard tool for assessing risk.

The Revised IPSS (IPSS-R) uses the same categories (cytogenetics, blast count and number of cytopenias), however it refines the subgroups to give an improved prediction of survival and risk of evolution to AML.

The IPSS-R is the preferred scoring system to determine prognosis at diagnosis. The IPSS-R is not designed as a dynamic system and therefore cannot be used to assess prognosis at other time points. The WHO based classification system (WPSS) is designed as a dynamic system and can be used at any time during the disease course.

CMML has a separate prognostic scoring system.

In This Section

IPSS-R

WPSS

IPSS

IPSS-R

  1. Sum the scores for each factor (karyotype, % bone marrow blasts, level of cytopenia).

     

    Score

    Karyotype

    Bone marrow blast%

    Haemoglobin concentration (g/L)

    Platelet count (x109/L)

    Neutrophil count

    (x109/L)

    0

    Very good

    ≤ 2

    ≥ 100

    ≥ 100

    ≥ 0.8

    0.5

    50 to < 100

    < 0.8

    1

    Good

    > 2 to < 5

    80 to < 100

    < 50

    1.5

    < 80

    2

    Intermediate

    5 to 10

    3

    Poor

    > 10

    4

    Very poor

  2. Use the summed scores to determine risk category and prognosis.

     

    Score

    Risk Category

    Median OS (years)

    25% AML evolution

    (median years)

    ≤ 1.5

    Very low

    8.8

    NR

    > 1.5 to 3

    Low

    5.3

    10.8

    > 3 to 4.5

    Intermediate

    3.0

    3.2

    > 4.5 to 6

    High

    1.6

    1.4

    > 6

    Very high

    0.8

    0.73

IPSS-R calculator

Greenberg, Tuechler, Schanz et al, Revised International Prognostic Scoring System (IPSS-R) for Myelodysplastic Syndrome. Blood 120: 2454, 2012.

WPSS

  1. Sum the scores for each factor (WHO category, karyotype, transfusion requirement).

     

    Score

    WHO Category

    Karyotype

    Transfusion requirement *

    0

    RA, RARS, 5q-

    Good

    None

    1

    RCMD

    Intermediate

    Regular

    2

    RAEB-1

    Poor

    3

    RAEB-2

    * Transfusion dependency is defined as at least one red transfusion every 8 weeks over a period of 4 months.

  2. Use summed scores to determine risk category and prognosis.

     

    Score

    Risk Category

    Median OS (months)

    2 month probability of

    progression to AML

    0

    Very low

    141

    3%

    1

    Low

    66

    6%

    2

    Intermediate

    48

    21%

    3 to 4

    High

    26

    38%

    5 to 6

    Very high

    9

    80%

    WPSS scoring system

    Malcovati L, Germing U, Kuendgen A, et al. Time-dependent prognostic scoring system for predicting survival and leukemic evolution in myelodysplastic syndromes. J Clin Oncol 2007; 25:3503.

IPSS

The IPSS scoring system has been replaced by the updated IPSS-R score of prognosis. However, PHARMAC uses the IPSS categories of "intermediate-2" and "high-risk" in the eligibility criteria for azacitidine.

Prognostic Variable

Score Value

BM Blasts (%)

Karyotype

Cytopenias

0

<5%

Good

0/1

0.5

5-10%

Intermediate

2/3

1.0

 

Poor

 

1.5

11-20%

 

 

2.0

21-30%

 

 

Scores for risk groups are as follows:

CMML Score

In general the prognosis of CMML is poor. There are a number of staging systems available which incorporate clinical findings, laboratory results and some include cytogenetic / molecular testing. The IPSS-R is used although CMML is not strictly classified as MDS. No single scoring system has been shown to be superior and two options are given here.

 

Overall survival

Dysplasitic type

Proliferative type

CMML-0

31

48

17

CMML-1

19

29

15

CMML-2

13

*

*

Median survival is given in months.

* Difference in survival for CMML-2 subgroups was not statistically significant.

Schuler, E., et al. (2014). "Refined medullary blast and white blood cell count based classification of chronic myelomonocytic leukemias." Leukemia Research 38(12): 1413-1419.

MAYO prognostic model

This is based on peripheral blood findings – absolute monocyte count, presence of immature cells, anaemia and thrombocytopenia. One point is allocated for each feature present.

Patnaik, M. M., et al. (2013). "Mayo prognostic model for WHO-defined chronic myelomonocytic leukemia: ASXL1 and spliceosome component mutations and outcomes." Leukemia 27(7): 1504-1510.

Further reading

Itzykson, R., et al. (2013). "Prognostic Score Including Gene Mutations in Chronic Myelomonocytic Leukemia." Journal of Clinical Oncology 31(19): 2428-2436.

About this Canterbury DHB document (4957):

Document Owner:

Emma-Jane McDonald (see Who's Who)

Issue Date:

June 2017

Next Review:

June 2019

Keywords:

Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer

Topic Code: 4957