Table 1.

Classifications of MDS and premalignant cytopenias

ClassificationMDS subtypes or related conditions
2016 WHO classification (revised fourth edition) • MDS with single-lineage dysplasia (MDS-SLD)
• MDS with multilineage dysplasia (MDS-MLD)
• MDS with ring sideroblasts, with single lineage dysplasia (MDS-RS-SLD) or multilineage dysplasia (MDS-RS-MLD)
• MDS with isolated del(5q) (MDS del (5q))
• MDS with excess blasts (MDS-EB), type 1 (MDS-EB-1) or type 2 (MDS-EB-2)
• MDS, unclassifiable (MDS-U) 
International Consensus Classification (2022)17  MDS subtypes
• MDS with mutated SF3B1 (MDS-SF3B1)
• MDS with del(5q) (MDS del(5q))
• MDS, NOS—without dysplasia
• MDS, NOS—with single-lineage dysplasia
• MDS, NOS—with multilineage dysplasia
• MDS with excess blasts (MDS-EB)
• MDS with mutated TP53
• MDS/AML 
Related clonal disorders
• Clonal hematopoiesis of indeterminate potential (CHIP)
• Clonal cytopenia of undetermined significance (CCUS)
• VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome: autoinflammatory syndrome associated with anemia and clonal hematopoiesis due to somatic mutation in the UBA1 gene 
2022 WHO classification (fifth edition)20  MDS with defining genetic abnormalities
• MDS with low blasts and isolated 5q deletion (MDS-5q)
• MDS with low blasts and SF3B1 mutation (MDS-SF3B1)
• MDS with biallelic TP53 inactivation (MDS-bi TP53)
MDS, morphologically defined
• MDS with low blasts (MDS-LB)
• MDS, hypoplastic (MDS-h)
• MDS with increased blasts (MDS-IB, including MDS with fibrosis) 
ClassificationMDS subtypes or related conditions
2016 WHO classification (revised fourth edition) • MDS with single-lineage dysplasia (MDS-SLD)
• MDS with multilineage dysplasia (MDS-MLD)
• MDS with ring sideroblasts, with single lineage dysplasia (MDS-RS-SLD) or multilineage dysplasia (MDS-RS-MLD)
• MDS with isolated del(5q) (MDS del (5q))
• MDS with excess blasts (MDS-EB), type 1 (MDS-EB-1) or type 2 (MDS-EB-2)
• MDS, unclassifiable (MDS-U) 
International Consensus Classification (2022)17  MDS subtypes
• MDS with mutated SF3B1 (MDS-SF3B1)
• MDS with del(5q) (MDS del(5q))
• MDS, NOS—without dysplasia
• MDS, NOS—with single-lineage dysplasia
• MDS, NOS—with multilineage dysplasia
• MDS with excess blasts (MDS-EB)
• MDS with mutated TP53
• MDS/AML 
Related clonal disorders
• Clonal hematopoiesis of indeterminate potential (CHIP)
• Clonal cytopenia of undetermined significance (CCUS)
• VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome: autoinflammatory syndrome associated with anemia and clonal hematopoiesis due to somatic mutation in the UBA1 gene 
2022 WHO classification (fifth edition)20  MDS with defining genetic abnormalities
• MDS with low blasts and isolated 5q deletion (MDS-5q)
• MDS with low blasts and SF3B1 mutation (MDS-SF3B1)
• MDS with biallelic TP53 inactivation (MDS-bi TP53)
MDS, morphologically defined
• MDS with low blasts (MDS-LB)
• MDS, hypoplastic (MDS-h)
• MDS with increased blasts (MDS-IB, including MDS with fibrosis) 

NOS, not otherwise specified.

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