Criteria
Molecular International Prognostic Scoring System (IPSS-M) risk assessment[62]
A refined version of the IPSS and IPSS-R that combines somatic mutations (31 genes) with haematological and cytogenetic parameters to risk stratify patients with MDS. The IPSS-M classifies MDS patients into the following six risk groups based on a risk score derived from clinical (bone marrow blast percentage, platelet count, haemoglobin level), cytogenetic, and genetic prognostic factors:
Very low risk (risk score: ≤-1.5)
Low risk (risk score: >-1.5 to -0.5)
Moderate low risk (risk score: >-0.5 to 0)
Moderate high risk (risk score: >0 to 0.5)
High risk (risk score: >0.5 to 1.5)
Very high risk (risk score: >1.5).
The risk score and risk groups can be calculated using a web-based tool: IPSS-M risk calculator Opens in new window
Revised International Prognostic Scoring System (IPSS-R) risk assessment[63]
A refined version of the IPSS, using a larger patient database (Revised IPSS [IPSS-R], n = 7012, IPSS, n = 816) for analysis. Multiple statistically weighted clinical features were used to generate prognostic indicators.
Bone marrow blasts
≤2% = 0 points
>2% to 5% = 1 point
5% to 10% = 2 points
>10% = 3 points.
Karyotype (cytogenetic testing)
Very good risk: -Y, del(11q) = 0 points
Good risk: normal, del(5q), del(12p), del(20q), double including del(5q) = 1 point
Intermediate risk: del(7q), +8, +19, i(17q), any other single or double independent clones = 2 points
Poor risk: -7, inv(3)/t(3q)/del(3q), double including -7/del(7q), or complex 3 abnormalities = 3 points
Very poor risk: complex >3 abnormalities = 4 points.
Haemoglobin (g/dL)
≥10 = 0 points
8 to <10 = 1 point
<8 = 1.5 points.
Platelets (x 10⁹/L)
≥100 = 0 points
50 to <100 = 0.5 points
<50 = 1 point.
Absolute neutrophil count (x 10⁹/L)
≥0.8 = 0 points
<0.8 = 0.5 points.
Total summed score:
<1.5 points = very low risk
>1.5 to 3.0 points = low risk
>3.0 to 4.5 points = intermediate risk
>4.5 to 6.0 points = high risk
>6 points = very high risk.
A web-based IPSS-R calculator tool is available. MDS Foundation: Revised International Prognostic Scoring System (IPSS-R) for myelodysplastic syndromes risk assessment calculator Opens in new window
The World Health Organization Prognostic Scoring System (WPSS)[64]
Unlike prognostic scoring systems that predict outcomes based upon findings at diagnosis, the WPSS provides prognostic information for patients with myelodysplastic syndrome (MDS) at any time during the course of their disease.
The World Health Organization (WHO) category
Refractory anaemia; refractory anaemia with ringed sideroblasts; MDS with isolated del(5q) and marrow blasts <5% = 0 points
Refractory cytopenia with multilineage dysplasia; refractory cytopenia with multilineage dysplasia and ringed sideroblasts = 1 point
Refractory anaemia with excess of blasts-1 = 2 points
Refractory anaemia with excess of blasts-2 = 3 points.
The WHO classification for MDS was revised in 2016.[65] In the revised classification, the terms 'refractory anaemia' and 'refractory cytopenia' have been replaced with the term 'MDS'.
Karyotype (cytogenetic testing)
Good risk: normal, -Y, del(5q), del(20q) = 0 points
Intermediate risk: not classified as good risk or poor risk (other abnormalities) = 1 point
Poor risk: complex (≥3 abnormalities), chromosome 7 anomalies = 2 points.
RBC transfusion requirement
None = 0 points
Regular = 1 point (transfusion dependency defined as having at least one RBC transfusion every 8 weeks over a period of 4 months).
Total summed score:
0 points = very low risk
1 point = low risk
2 points = intermediate risk
3-4 points = high risk
5-6 points = very high risk.
International Prognostic Scoring System (IPSS) risk assessment[66]
Scores for three domains (bone marrow blasts, karyotype, and peripheral blood cytopenias) are summed to give an overall score and assign a risk category; age (≤60 years) is also important.
Bone marrow blasts
<5% = 0 points
5% to 10% = 0.5 points
NA = 1 point
11% to 20% = 1.5 points
21% to 30% = 2 points.
Karyotype (cytogenetic testing)
Good risk: normal, isolated-Y, isolated del(5q), or isolated del(20q) = 0 points
Intermediate risk: all karyotypes not defined as good or poor = 0.5 points
Poor risk: abnormal chromosome 7, or a complex karyotype (3 or more abnormalities) = 1 point.
Peripheral blood cytopenias
0 or 1 = 0 points
2 or 3 = 0.5 points.
Total summed score:
0 points = low risk
0.5 to 1.0 points = intermediate-1 (INT-1) risk
1.5 to 2.0 points = INT-2 risk
≥2.5 points = high risk.
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