Table 1 Main Differences and similarities between ICC and WHO-4 and -5 classifications excluding CMML [3, 6, 7].

From: Defining remission and relapse after allogeneic hematopoietic cell transplantation in myelodysplastic/myeloproliferative neoplasms and optimization of transplantation outcomes: Recommendations from the EBMT practice harmonisation and guidelines committee

WHO-4

WHO-5

ICC

Differences

Atypical CML, BCR-ABL1negative

MDS/MPN with neutrophilia (MDS/MPN-N)

Atypical CML (aCML)

Significant terminology changes in WHO 5ED; ICC deleted reference to the lack of BCR::ABL1 gene fusion in the name. Criteria essentially unchanged from revised WHO 4ED

MDS/MPN with ring sideroblasts and thrombocytosis

MDS/MPN with SF3B1 mutation and thrombocytosis (MDS/MPN-SF3B1-T)

A. MDS/MPN with thrombocytosis and SF3B1mutation (MDS/MPN-SF3B1-T)

B. MDS/MPN with ring sideroblasts and thrombocytosis, NOS (MDS/MPN-RS-T, NOS)

Disease now requires presence of an SF3B1 mutation.

The ICC also recognizes a separate category – 'SF3B1 negative' cases defined by ring sideroblasts and thrombocytosis. There is limited outcome data on this subset

MDS/MPN, U

MDS/MPN, NOS

MDS/MPN, NOS

Both made minor terminology change (“NOS” instead of “unclassifiable”). Criteria are unchanged from WHO 4ED

Not recognized

Not recognized

Clonal cytopenia with monocytosis of undetermined

significance (CCMUS)

Only ICC classification recognizes this category; considered within CCUS in WHO 5ED

Not recognized

Not recognized

Clonal monocytosis of undetermined significance (CMUS)

Only ICC classification recognizes this category

Not recognized

Not recognized

MDS/MPN with isochromosome (17q) (MDS/MPN-i(17q))

Only ICC classification recognizes this category (provisional entity within MDS/MPN-NOS)