Major criteria:
(1) atypical megakaryocytic proliferation and one of the following:
(1a) reticulin and/or collagen fibrosis
(1b) hypercellular marrow with granulocytic proliferation, usually with decreased erythropoiesis
(2) one or both of the following:
(2a) presence of JAK2 mutation (JAK2 617V>F) or other clonal marker (MPL515W>L/K)
(2b) exclusion of marrow fibrosis secondary to infection, autoimmune disease, metastatic carcinoma, hairy cell leukemia, or other cause
(3) exclusion of other hematologic disorders (CML, myelodysplasia, polycythemia vera, etc)
where:
• The megakaryocytes may range in size. They show an abnormal nuclear to cytoplasmic ratio. Nuclei are hyperchromatic, bulbous or irregularly folded.
• Absence of BCR-ABL is used to exclude CML.
Minor criteria:
(1) leukoerythroblastosis
(2) elevated serum LDH concentration
(3) anemia
(4) palpable splenomegaly
|
Number of Major Criteria
|
Number of Minor Criteria
|
Diagnosis of PV
|
|
3
|
>=2
|
definite
|
