2016 WHO myelodysplastic syndrome subtypes

Name	Dysplastic lineages	Cytopenias*	Ring sideroblasts as % of marrow erythroid elements	BM and PB blasts	Cytogenetics by conventional karyotype analysis
MDS with single lineage dysplasia (MDS-SLD)	1	1 or 2	<15%/<5%^¶	BM <5%, PB <1%, no Auer rods	Any, unless fulfills all criteria for MDS with isolated del(5q)
MDS with multilineage dysplasia (MDS-MLD)	2 or 3	1 to 3	<15%/<5%^¶	BM <5%, PB <1%, no Auer rods	Any, unless fulfills 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 or 2	≥15%/≥5%^¶	BM <5%, PB <1%, no Auer rods	Any, unless fulfills all criteria for MDS with isolated del(5q)
MDS-RS with multilineage dysplasia (MDS-RS-MLD)	2 or 3	1 to 3	≥15%/≥5%^¶	BM <5%, PB <1%, no Auer rods	Any, unless fulfills all criteria for MDS with isolated del(5q)
MDS with isolated del(5q)	1 to 3	1 to 2	None or any	BM <5%, PB <1%, no Auer rods	del(5q) alone or with 1 additional abnormality except –7 or del(7q)
MDS with excess blasts (MDS-EB)
MDS-EB-1	0 to 3	1 to 3	None or any	BM 5 to 9% or PB 2 to 4%, no Auer rods	Any
MDS-EB-2	0 to 3	1 to 3	None or any	BM 10 to 19% or PB 5 to 19% or Auer rods	Any
MDS, unclassifiable (MDS-U)
With 1% blood blasts	1 to 3	1 to 3	None or any	BM <5%, PB = 1%,^Δ no Auer rods	Any
With single lineage dysplasia and pancytopenia	1	3	None or any	BM <5%, PB <1%, no Auer rods	Any
Based on defining cytogenetic abnormality	0	1 to 3	<15%^◊	BM <5%, PB <1%, no Auer rods	MDS-defining abnormality
Refractory cytopenia of childhood	1 to 3	1 to 3	None	BM <5%, PB <2%	Any

BM: bone marrow; PB: peripheral blood; MDS: myelodysplastic syndrome
* 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. Peripheral blood monocytes must be <1 × 10⁹/L.
¶ If SF3B1 mutation is present.
Δ One percent peripheral blood 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.

Republished with permission of the American Society of Hematology, from Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016; 127:2391. Copyright © 2016; permission conveyed through Copyright Clearance Center, Inc.

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2016 WHO myelodysplastic syndrome subtypes