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Myelodysplastic syndromes (MDS) are a group of heterogenous disorders, making a single unified prognostic model difficult to implement. In recent years, many prognostic models have been forwarded with varying use globally. As part of our editorial theme ‘Diagnosis and prognosis in MDS’, we summarize the use of different prognostic scoring systems in the clinical setting. These were discussed by Rami Komrokji at the European School of Haematology (ESH) 8th Translational Research Conference: Myelodysplastic Syndromes.1
Komrokji1 presented real-world data on several key prognostic scoring systems, highlighting several factors that make an ideal prognostic model (Figure 1).
Figure 1. Key prognostic scoring systems and ideal prognostic characteristics*
IPSS, International Prognostic Scoring System; MDACC, MD Anderson Cancer Center; WPSS, World Health Organization Classification-Based Prognostic Scoring System.
*Adapted from Komrokji1
The IPSS-R incorporates more cytopenia subgroups, further refinement of blast count by splitting the low marrow blast percentage value, and importantly, provides cytogenetic risk stratification. The possible range of summed scores is 0–10 and parameters are summarized in Table 1.
Table 1. IPSS-R scoring system*
Parameter |
IPSS-R score |
||||
---|---|---|---|---|---|
Cytogenetic risk |
Very good |
Good |
Intermediate |
Poor |
Very poor |
0 |
1 |
2 |
3 |
4 |
|
Marrow blasts |
≤2% |
>2% to ≤5% |
5–10% |
>10% |
— |
0 |
1 |
2 |
3 |
— |
|
Hemoglobin |
≥10 |
8 to <10 |
<8 |
— |
— |
0 |
1 |
1.5 |
— |
— |
|
Platelet count |
>100 |
50 to <100 |
<50 |
— |
— |
0 |
0.5 |
1 |
— |
— |
|
Absolute neutrophil count (× 109/L) |
≥0.8 |
<0.8 |
— |
— |
— |
0 |
0.5 |
— |
— |
— |
|
IPSS-R, Revised International Prognostic Scoring System. |
Benefits
Challenges
Real-world data
Table 2. WPSS scoring system*
Parameter |
Score value |
|||
---|---|---|---|---|
0 |
1 |
2 |
3 |
|
WHO category |
RA, RARS, del(5q) |
RCMD, RCMD-RS |
RAEB-1 |
RAEB-2 |
Karyotype |
Good |
Intermediate |
Poor |
— |
Transfusion requirement |
No |
Regular |
— |
— |
RA, refractory anemia; RAEB, refractory anemia with excess blasts; RARS, refractory anemia with ringed sideroblasts; RCMD, refractory cytopenia with multilineage dysplasia; RCMD-RS, refractory cytopenia with multilineage dysplasia and ringed sideroblasts; WHO, World Health Organization. |
Benefits
Challenges
Table 3. Global MDACC scoring system*
Prognostic category |
Score value |
||
---|---|---|---|
1 |
2 |
3 |
|
Performance status |
— |
≥2 |
— |
Age, years |
60–64 |
≥65 |
— |
Platelets, × 109/L |
50–199 |
30–49 |
<30 |
Hemoglobin, g/dL |
— |
<12 |
— |
BM blasts, % |
5–10 |
11–29 |
— |
WBC, × 109/L |
— |
>20 |
— |
Cytogenetics |
— |
— |
Chromosome 7 abnormality (≥3 abnormalities) |
Prior transfusion |
Yes |
— |
— |
BM, bone marrow; MDACC, MD Anderson Cancer Center; WBC, white blood cell. |
Benefits
Challenges
Table 4. Lower risk MDACC Model (LR-PSS) scoring system*
Parameter |
LR-PSS score |
|
---|---|---|
1 |
2 |
|
Cytogenetics |
Not normal or del(5q) |
— |
Hemoglobin, g/dL |
<10 |
— |
Platelets, × 109/L |
50–200 |
<50 |
BM blasts, % |
≥4 |
— |
BM, bone marrow; LR PSS, lower risk MDACC Model; MDACC, MD Anderson Cancer Center. |
Study validation
Having a unified model should be the goal for prognosis in MDS; therefore, it is important to compare the prognostic value of these models.
Komrakji1 highlighted that all current risk stratification models used in MDS can predict clinical outcomes for patients; however, further personalization is needed owing to the heterogeneity of the disease. This will include dividing patients by those with excess blasts versus without, integrating more genomic and clinical variables, and thinking of patient-related factors.
The new IPSS system, integrating molecular information, is to be introduced to clinical practice soon. However, issues around global access to molecular profiling may mean IPSS-R is still required in certain regions, as detailed in the video below.
Can IPSS-R be used as a prognostic tool in HSCT in regions where molecular analysis is unavailable?
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