Myeloproliferative neoplasms are abnormal proliferations of one or more terminal myeloid cell lines in the peripheral blood. They are characterised by extramedullary hematopoiesis, myelofibrosis, and transformation to acute leukaemia.
Myeloproliferative neoplasms
| Chronic myeloid leukaemia | Polycythaemia vera | Essential thrombocythaemia | Primary myelofibrosis | |
|---|---|---|---|---|
| WBC | Marked elevation | Normal/Increased | Normal/Increased | Variable |
| Hct | Normal/decreased | Marked elevation | Normal | Decreased |
| PLT | Varibale | Normal or increased | Marked increase | Variable |
| Mutation | bcr-abl | JAK2 | JAK2, CALR, MPL | JAK2, CALR, MPL |
| Signs and symptoms | – | Erythromelalgia, aquagenic pruritus, low EPO | Erythromelalgia, large platelets | Hepatosplenomegaly, dry tap, tear drop cells |
| Treatment | Imatinib | Phelbotomy, Hydroxyurea, Aspirin, Ruxolitinib | Hydroxyurea, Aspirin, Anagrelide | Bone marrow transplant, Splenectomy, Ruxolitinib |
Some myeloproliferative neoplasms and their characteristic mutations
| Neoplasm | Mutation |
|---|---|
| Chronic myeloid leukaemia | bcr-abl |
| Polycythemia vera | JAK2 |
| Essential thrombocythaemia and Primary myelofibrosis | JAK2, CALR, MPL |
- WHO classification of myeloproliferative neoplasms (MPN)
- Chronic Myeloid Leukemia (CML), BCR-ABL1+
- Chronic neutrophilic leukemia (CNL)
- Polycythemia Vera (PV)
- Primary Myelofibrosis (PMF)
- PMF, pre-fibrotic/early stage
- PMF, overt fibrotic stage
- Essential thrombocythemia (ET)
- Chronic eosinophilic leukemia, not otherwise specified (NOS)
- MPN, unclassifiable
- Mastocytosis