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Table 1 A summary of the 2016 World Health Organisation (WHO) diagnostic criteria

From: Molecular pathogenesis of the myeloproliferative neoplasms

Disease Major criteria Minor criteria Diagnosis Reference
Polycythaemia vera 1. Hb > 16.5 g/dL (M) 16.0 g/dL (F) or, haematocrit > 49% (M) 48% (Female) or, increased red cell mass (> 125%)a
2. Bone marrow biopsy with characteristic morphology
3. Presence of JAK2 V617F or JAK2 exon 12 mutation
1. Serum erythropoietin level below normal All 3 major criteria or, Top 2 major and the minor criteria [1]
Essential Thrombocythaemia 1. Platelet count > 450 × 109/L
2. Bone marrow biopsy with characteristic morphology
3. Not meeting criteria for another MPN/myeloid neoplasm
4. Presence of JAK2, CALR, or MPL
1. Presence of another clonal marker or absence of evidence for a reactive thrombocytosis All 4 major criteria or, Top 3 major and the minor criteria [1]
Pre-fibrotic primary myelofibrosis 1. Bone marrow biopsy with characteristic morphology without reticulin fibrosis > grade 1
2. Not meeting criteria for another MPN/myeloid neoplasm
3. Presence of JAK2, CALR or MPL mutation, or, another clonal marker, or, no identifiable cause of reactive fibrosis
1. Anaemia not caused by a co-morbid condition
2. Leukocytosis ≥ 11 × 109/L
3. Palpable Splenomegaly
4. Lactate dehydrogenase above upper limit of normal
All 3 major criteria plus at least one minor criteria (confirmed on two separate measurements) [1]
Myelofibrosis 1. Bone marrow biopsy with characteristic morphology with either reticulin or collagen fibrosis grades 2 or 3
2. Not meeting criteria for another MPN/myeloid neoplasm
3. Presence of JAK2, CALR or MPL mutation, or, another clonal marker, or, no identifiable cause of reactive fibrosis
1. Anaemia not caused by a co-morbid condition
2. Leukocytosis ≥ 11 × 109/L
3. Palpable Splenomegaly
4. Lactate dehydrogenase above upper limit of normal
5. Leukoerythroblastosis
All 3 major criteria plus at least one minor criteria (confirmed on two separate measurements) [1]
Blast phase MPN Patients with MPN and peripheral or bone marrow myeloid blast percentage > 20%   Major criteria met [11]
  1. A summary of the 2016 WHO criteria for the distinct clinical entities of PV, ET, Pre-PMF, PMF. Consensus diagnostic criteria for blast phase transformation are included. A minority of patients with a diagnosis of a MPN disorder may not meet diagnostic criteria for any of these distinct entities of any other myeloid neoplasm and may be classed as MPN unclassifiable [1]
  2. aBritish Society of Haematology guidelines propose higher haematocrit levels of > 52% in males and > 48% in females [12]