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2016 revision to WHO classification of MDS

* Cytopenias defined as: hemoglobin <10 g/dL; platelet count <100 × 109/L; and absolute neutrophil count <1.8 × 109/L. Rarely, MDS may present with mild anemia or thrombocytopenia above these levels. PB monocytes must be <1 × 109/L

† If SF3B1 mutation is present

‡ One percent PB blasts must be recorded on at least 2 separate occasions.

§ Cases with ≥15% ring sideroblasts by definition have significant erythroid dysplasia, and are classified as MDS-RS-SLD.

¥ Unbalanced: Loss of chromosome 7 or del(7q), del(5q), isochromosome 17q or t(17p), loss of chromosome 13 or del(13q), del(11q), del(12p) or t(12p), del(9q), idic(X)(q13). Balanced: t(11;16)(q23.3;p13.3), t(3;21)(q26.2;q22.1), t(1;3)(p36.3;q21.2), t(2;11)(p21;q23.3), inv(3)(q21.3q26.2)/t(3;3)(q21.3;q26.2), t(6;9)(p23;q34.1).

Disease Number of dysplastic lineages Number of cytopenias * Ring sideroblasts as percentage of marrowerythroid elements Bone marrow (BM) and peripheral blood (PB) blasts Cytogenetics by conventional karyotype analysis

MDS with single lineage dysplasia (MDS-SLD)

1

1-2

< 15% / < 5% †

BM < 5%, PB < 1%, no Auer rods

Any, unless fulfils all criteria for MDS with isolated del(5q)

MDS with multilineage dysplasi (MDS-MLD)

2 - 3

1 - 3

< 15% / < 5% †

BM < 5%, PB < 1%, no Auer rods

Any, unless fulfils all criteria for MDS with isolated del(5q)

MDS with ring sideroblasts (MDS-RS)

MDS-RS with single lineage dysplasia (MDS-RS-SLD)

1

1 - 2

≥ 15% / ≥ 5% †

BM < 5%, PB < 1%, no Auer rods

Any, unless fulfils all criteria for MDS with isolated del(5q)

MDS-RS with multilineage dysplasia (MDS-RS-MLD)

2 - 3

1 - 3

≥ 15% / ≥ 5% †

BM < 5%, PB < 1%, no Auer rods

Any, unless fulfils all criteria for MDS with isolated del(5q)

MDS with isolated del(5q)

1 - 3

1 - 2

None or any

BM < 5%, PB < 1%, no Auer rods

del(5q) alone or with 1 additional abnormality, except loss of chromosome 7 or del(7q)

MDS with excess blast

MDS-EB-1

0 - 3

1 - 3

None or any

BM 5% - 9% or PB 2% - 4%, no Auer rods

Any

MDS-EB-2

0 - 3

1 - 3

None or any

BM 10% - 19% or PB 5% - 19% or Auer rods

Any

MDS, unclassifiable (MDS-U)

MDS-U with 1% blood blasts

1 - 3

1 - 3

None or any

BM <5%, PB =1% ‡, no Auer rods

Any

MDS-U with single lineage dysplasia and pancytopenia

1

3

None or any

BM <5%, PB <1%, no Auer rods

Any

MDS-U based on defining cytogenetic abnormality

0

1 - 3

<15% §

BM <5%, PB <1%, no Auer rods

MDS-defining abnormality ¥