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| Early/pre-fibrotic stage PMF (pre-PMF) |
Overt fibrotic stage PMF |
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| Major Criteria: |
Major Criteria: |
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| Proliferation of atypical megakaryocytes, accompanied by marrow fibrosis < grade 2*, increased age-adjusted BM cellularity, granulocytic proliferation, but without reticulin fibrosis, and decreased erythropoiesis. |
Proliferation of atypical megakaryocytes, accompanied by either reticulin and/or collagen fibrosis grade 2 or 3*, osteosclerosis, and extramedullary hematopoiesis. |
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| Criteria for BCR-ABL1, CML, ET, PV, PMF, MDS or other myeloid neoplasms not met. |
Criteria for BCR-ABL1, CML, ET, PV, PMF, MDS or other myeloid neoplasms not met. |
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| Presence of JAK2, CALR or MPL mutations, or in the absence of these, presence of another clonal marker (e.g., ASXL1, EZH2, TET2, IDH1/IDH2, SRSF2, SF3B1 mutations) or absence of reactive MF. |
Presence of JAK2, CALR or MPL mutations, or in the absence of these, presence of another clonal marker (e.g., ASXL1, EZH2, TET2, IDH1/IDH2, SRSF2, SF3B1 mutations) or absence of reactive MF. |
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| Minor Criteria: |
Minor Criteria: |
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| Presence of at least one of the following: |
Presence of at least one of the following: |
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| Anemia |
Anemia |
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| Leukocytosis ≥ 11×109/L. |
Leukocytosis ≥ 11×109/L. |
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| Palpable splenomegaly. |
Palpable splenomegaly. |
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| Increased LDH. |
Increased LDH. |
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| Requirements for diagnosis: |
Leukoerythroblastosis. |
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| All 3 major criteria + at least 1 minor criterion |
Requirement for diagnosis: |
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All 3 major criteria + at least 1 minor criterion |
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*PMF shows a higher degree of megakaryocytic atypia than in any other MPN subtype, with distinctive features including small to giant megakaryocytes with cloud-like, hypolobulated, and hyperchromatic nuclei, and the presence of abnormal large dense clusters of >6 megakaryocytes.
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