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 × 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. | ||
| 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 × 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. | ||
Abbreviations: PB, peripheral blood; BM, bone marrow; abn, abnormality
References 3,4.
Reference 21.
Reference 30.
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.
IPSS Types of Cytopenia28 : Hemoglobin <10g per deciliter; Absolute neutrophil count <1500 per cubic millimeter; Platelet count < 100,000 per cubic millimeter.