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] |