Table 1. MDS Classification
Systems.1
FAB Classification
System2
|
WHO Classification
System3
|
IPSS Risk-Based Classification
System4
|
|
|
Refractory Anemia (RA): Cytopenia of one PB lineage; normo- or
hypercellular marrow with dysplasias; < 1% PB Blasts and < 5% BM Blasts. |
Myelodysplastic Syndromes |
Overall IPSS Risk Score Based On:
|
|
Refractory Anemia (RA) |
Marrow Blast Percentage
|
|
With ringed sideroblasts (RARS) |
Blast % |
IPSS Score |
|
Refractory Anemia with Ringed Sideroblasts (RARS): Cytopenia, dysplasia
and the same % blast involvement in BM and PB as RA. Ringed sideroblasts
account for >15% of nucleated cells in marrow. |
Without ringed sideroblasts |
< 5 |
0 |
|
|
5-10 |
0.5 |
|
Refractory Cytopenia (MDS) with Multilineage Dysplasia (RCMD) |
11-20 |
1.5 |
|
|
21-30 |
2.0 |
|
Refractory Anemia with Excess Blasts (RAEB): Cytopenia of two or more
PB lineages; dysplasia involving all 3 lineages; < 5% PB blasts and 5-20%
BM Blasts. |
Refractory Anemia with Excess Blasts (RAEB) |
Cytogenetic
Features5
|
|
|
Karyotype |
IPSS Score |
|
5q-Syndrome |
Good prognosis (-Y, 5q-,20q-) |
0 |
|
|
Intermediate prognosis |
0.5 |
|
Myelodysplastic syndrome, unclassifiable |
Poor prognosis (abn. 7; Complex) |
1.0 |
|
Refractory Anemia with Excess Blasts in Transformation (RAEB-T):
Hematologic features identical to RAEB. > 5% Blasts in PB or 21-30% Blasts
in BM or the presence of Auer rods in the blasts. |
Myelodysplastic/Myeloproliferative Diseases |
Cytopenias6
|
|
|
Cytopenia |
IPSS Score |
|
|
None or 1 Type |
0 |
|
Chronic Myelomonocytic |
2 or 3 Types |
0.5 |
|
Chronic Myelomonocytic Leukemia (CMML): Monocytosis in PB (>1
x 109 per liter); <5% blasts in PB and up to 20% BM blasts |
Leukemia (CMML) |
|
|
|
Atypical Chronic Myelogenous |
Overall IPSS Score and Survival
|
|
Leukemia (aCML) |
Overall Score |
Median Survival |
|
|
Low (0) |
5.7 Yrs. |
|
Juvenile Myelomonocytic |
|
Intermediate |
|
|
Leukemia (JMML) |
1 (0.5 or 1.0) |
3.5 Yrs |
|
|
2 (1.5 or 2.0) |
1.2 Yrs. |
|
|
High ( 2.5) |
0.4 Yrs. |
|
1 Abbreviations: PB, peripheral blood; BM, bone marrow; abn, abnormality
2 References
3,4.
3 Reference 21.
4 Reference 30.
5 IPSS Cytogenetic
Classification28:
Good prognosis: -Y only, normal, del(5q) only, del(20q) only; Intermediate
prognosis: +8, Single miscellaneous abnormality, double abnormalities; Poor
prognosis: Complex (i.e. 3 abnormalities), any chromosome 7
abnormality.
6 IPSS Types of
Cytopenia28:
Hemoglobin <10g per deciliter; Absolute neutrophil count <1500 per cubic
millimeter; Platelet count < 100,000 per cubic millimeter.