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Hematology Journal Club Classification of myelodysplastic syndrome Title: Classification of myelodysplastic syndrome Presented by: Fatemeh Jamshidi

Myelodysplastic syndrome (MDS)

definition Myelodysplastic syndromes (MDS) are clonal hematopoietic stem cell disorders characterized by dysplastic, ineffective and neoplastic hematopoiesis. Transformation to AML is frequent.

characteristics 1 2 3 4 PB: Cytopenia BM: Hypercellular Dysplasia varying degree of tri-lineage cytopenia

incidence 70 years

clinical manifestations Anemia Infection bleeding

morphology Dyserythropoiesis Dysgranulopoiesis Dysmegakaryopoiesis

MDS classification history 2016 2008 WHO 1982 WHO FAB

FAB classification FAB subgroup PB blasts (%) BM Ring Sideroblasts (%) Monocytes (×10/L) RA < 1<1 ≤ 1 < 5 < 15 < 1 RARS ≤ 1 ≥ 15 RAEB 1 - 5 5 - 20 variable CMML < 20 > 1 RAEB-t ≥ 5 Auer rods 21 – 30

FAB Blasts (%) 20 – 30 ? Monocytes > 1000/ml ? RAEB-t Blasts (%) YES Monocytes > 1000/ml ? RAEB-t NO YES Blasts (%) 5 – 20 ? CMML NO YES RS (%) > 15 ? RAEB NO YES RA RARS

FAB WHO

FAB WHO × × RAEB-t CMML RAEB RA AML MDS/MPN RAEB-1 RAEB-2 RA RA RCUD RN RA RCMD RT

2008 WHO classification RCUD < 1 < 5 < 15 RARS _ ≥ 15 RCMD subgroup PB blasts (%) BM Ring Sideroblasts (%) Monocytes (×10³/L) RCUD < 1 < 5 < 15 RARS _ ≥ 15 RCMD RCMD-RS RAEB-1 5-9 RAEB-2 5-19 10-19 Auer rods Del (5q-) MDS-U

Del 5q- MDS(5q-) ? Auer rods RAEB-2 ? Blasts (%) RAEB-2 10 – 19 ? YES MDS(5q-) NO Auer rods ? YES RAEB-2 NO Blasts (%) 10 – 19 ? YES RAEB-2 NO Blasts (%) 5 – 9 ? YES RAEB - 1 YES RCMD-RS YES Multilineages ? NO RARS NO RS (%) > 15 ? YES RCMD Multilineages ? NO RA NO

2008 2016 WHO

2016 WHO classification WHO subgroup Dysplastic lineages cytopenias PB blasts (%) BM Ring sideroblasts (%) MDS-SLD 1 1 or 2 < 1 < 5 < 15 MDS-MLD 2 or 3 1 - 3 MDS-RS-SLD ≥ 15 MDS-RS-MLD MDS-del 5q 1 - 2 - MDS-EB-1 0 - 3 2 - 4 5 - 9 MDS-EB-2 5 – 19 Auer rods - 19 MDS-U RCC < 2

mutations

Ring sideroblasts

SF3B1??

Maturity of mRNA

snRNPs (snurps)

U2 snRNP

mutated sf3b1

mutated sf3b1 ABCB7 Ring Sideroblasts

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