WHO criteria for overt PMF
| WHO overt PMF criteria . |
|---|
| Major criteria |
| 1. Presence of megakaryocytic proliferation and atypia, accompanied by either reticulin and/or collagen fibrosis grades 2 or 3* |
| 2. Not meeting WHO criteria for ET, PV, BCR-ABL1+ CML, myelodysplastic syndromes, or other myeloid neoplasms |
| 3. Presence of JAK2, CALR, or MPL mutation or in the absence of these mutations, presence of another clonal marker,† or absence of reactive myelofibrosis‡ |
| Minor criteria |
| Presence of at least 1 of the following, confirmed in 2 consecutive determinations: |
| a. Anemia not attributed to a comorbid condition |
| b. Leukocytosis ≥11 × 109/L |
| c. Palpable splenomegaly |
| d. LDH increased to above upper normal limit of institutional reference range |
| e. Leukoerythroblastosis |
| Diagnosis of overt PMF requires meeting all 3 major criteria, and at least 1 minor criterion |
| WHO overt PMF criteria . |
|---|
| Major criteria |
| 1. Presence of megakaryocytic proliferation and atypia, accompanied by either reticulin and/or collagen fibrosis grades 2 or 3* |
| 2. Not meeting WHO criteria for ET, PV, BCR-ABL1+ CML, myelodysplastic syndromes, or other myeloid neoplasms |
| 3. Presence of JAK2, CALR, or MPL mutation or in the absence of these mutations, presence of another clonal marker,† or absence of reactive myelofibrosis‡ |
| Minor criteria |
| Presence of at least 1 of the following, confirmed in 2 consecutive determinations: |
| a. Anemia not attributed to a comorbid condition |
| b. Leukocytosis ≥11 × 109/L |
| c. Palpable splenomegaly |
| d. LDH increased to above upper normal limit of institutional reference range |
| e. Leukoerythroblastosis |
| Diagnosis of overt PMF requires meeting all 3 major criteria, and at least 1 minor criterion |
See Table 8.
In the absence of any of the 3 major clonal mutations, the search for the most frequent accompanying mutations (eg, ASXL1, EZH2, TET2, IDH1/IDH2, SRSF2, SF3B1) are of help in determining the clonal nature of the disease.
BM fibrosis secondary to infection, autoimmune disorder, or other chronic inflammatory conditions, hairy cell leukemia or other lymphoid neoplasm, metastatic malignancy, or toxic (chronic) myelopathies.