Table 4 Overt fibrotic primary myelofibrosis (overt PMF) (WHO refs. # [4,5,6,7] versus ICC ref. # [14]).
Year Major criteria | 2001 Jaffe ES et al. ref. # [4] | 2008 Swerdlow SH et al. ref. # [5] | 2017 Swerdlow SH et al. ref. # [6] | 2024 Akkari Y et al. ref.# [7] | 2022 Arber DA. et al. ref.# [14] |
|---|---|---|---|---|---|
Blood | Leukoerythroblastosis, prominent red blood cell poikilocytosis with dacrocytes | ||||
Bone marrow morphology | Reticulin and / or collagen fibrosis, decreased cellularity, dilated marrow sinuses with intraluminal hematopoiesis. Megakaryocytic proliferation and atypia (clustering of megakaryocytes, abnormally lobulated megakaryocytic nuclei, naked megakaryocytic nuclei). New bone formation (osteosclerosis) | Presence of megakaryocytic proliferation and atypia usually accompanied by either reticulin and /or collagen fibrosis | Presence of megakaryocytic proliferation and atypia, usually accompanied by either reticulin and /or collagen fibrosis | Megakaryocytic proliferation and atypia accompanied by reticulin and/or collagen fibrosis grades 2 or 3 | Bone marrow biopsy showing megakaryocytic proliferation and atypia, accompanied by reticulin and/or collagen fibrosis grades 2 or 3 |
Criteria of Exclusion | Not meeting WHO criteria for polycythemia vera, BCR::ABL1 positive chronic myeloid leukemia, myelodysplastic syndromes or other myeloid neoplasms | Not meeting WHO criteria for polycythemia vera, BCR::ABL1 positive chronic myeloid leukemia, myeloid dysplastic syndromes or other myeloid neoplasms | WHO criteria for essential thrombocythemia, polycythemia vera, BCR-ABL1 positive chronic myeloid leukemia, myelodysplastic syndrome, or other myeloid neoplasms are not met | Diagnostic criteria for ET, PV, BCR::ABL1 positive CML, myelodysplastic syndrome, or other myeloid neoplasms are not met | |
Clonal genetic abnormality | Demonstration of JAK2 V617F or other clonal marker (e.g. MPL W515K/L), or in the absence of a clonal marker, no evidence that the bone marrow fibrosis or other changes are secondary to infection, autoimmune disorder or other chronic inflammatory condition, hairy cell leukemia or other lymphoid neoplasm, metastatic malignancy, or toxic (chronic) myelopathies | Demonstration of JAK2 V617F or other clonal marker (e.g. MPL W515K/L), or in the absence of a clonal marker, no evidence that the bone marrow fibrosis or other changes are secondary to infection, autoimmune disorder or other chronic inflammatory condition, hairy cell leukemia or other lymphoid neoplasm, metastatic malignancy, or toxic (chronic) myelopathies | JAK2, CALR, or MPL mutation or presence of another clonal marker | JAK2, CALR, or MPL mutation or presence of another clonal marker or absence of reactive myelofibrosis | |
Minor Criteria | Hematologic parameters: moderate to marked anemia, low, normal, elevated white blood cell count, platelet count decreased, normal or elevated, moderate to marked splenomegaly or hepatomegaly | Leukoerythroblastosis Increase in serum lactate dehydrogenase level Anemia Splenomegaly | Leukoerythroblastosis Increase in serum lactate dehydrogenase level Anemia Splenomegaly | Presence of at least one of the following, confirmed in 2 consecutive determinations: Anemia not attributed to a comorbid condition Leukocytosis ≥ 11 × 109/ L Splenomegaly detected clinically and/or by imaging Lactate dehydrogenase level above the upper limit of the institutional reference range Leukoerythroblastosis | Anemia not attributed to a comorbid condition Leukocytosis ≥ 11 x 109/ L Palpable splenomegaly Lactate dehydrogenase level above the above the reference range Leukoerythroblastosis |
Diagnosis requires meeting criteria 1 and 2 and criteria of exclusion | Diagnosis requires meeting all 3 major and 2 minor criteria | Diagnosis requires meeting all 3 major and 2 minor criteria | The diagnosis of overt primary myelofibrosis requires that all 3 major criteria and at least 1 minor criterion are met | The diagnosis overt PMF requires all 3 major criteria and at least 1 minor criterion confirmed in 2 consecutive determinations |
