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Mutational Analysis of Driver and Non-driver Mutations of Philadelphia Chromosome-negative Myeloproliferative Neoplasms; Diagnosis and Recent Advances in Treatment
World Journal of Cancer and Oncology Research
| Vol 3, Issue 1
Table 3. Simplified 2022 WHO diagnosis criteria forpre-PMF and overt fibrotic PMF; Adapted from: [6, 104].
| Early/pre-fibrotic stage PMF (pre-PMF) | Overt fibrotic stage PMF |
| Major Criteria: | Major Criteria: |
| 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. |
| 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. |
| 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. |
| Minor Criteria: | Minor Criteria: |
| Presence of at least one of the following: | Presence of at least one of the following: |
| Anemia | Anemia |
| Leukocytosis ≥ 11×109/L. | Leukocytosis ≥ 11×109/L. |
| Palpable splenomegaly. | Palpable splenomegaly. |
| Increased LDH. | Increased LDH. |
| Requirements for diagnosis: | Leukoerythroblastosis. |
| All 3 major criteria + at least 1 minor criterion | Requirement for diagnosis: |
| All 3 major criteria + at least 1 minor criterion |
*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.