![]() 10 This study included a small number of patients (83 overt PV and 68 mPV) and did not compare mPV to ET patients with hematocrit levels within the same order of magnitude and thus failed to explain why mPV present sub-normal hematocrit and hemoglobin levels. and others showed that mPV could be identified using red blood cell mass (RCM) measurement 9 8 (masked PV presenting an increased RCM above 125%) in place of bone marrow histology. We previously showed that another proportion of JAK2+ ET patients have early-stage polycythemia when red blood cell measurement (RCM) is systematically measured. 6 5 If mPV is misdiagnosed as ET, therapy is based on anti-aggregating agents (AA) only whereas phlebotomy + AA or cytoreductive therapy + AA would be more adequate. 4 3 Distinguishing ET from mPV is impossible based on hemoglobin or hematocrit values but is of the utmost importance since patients with mPV may have a high risk of thrombosis. Recently masked PV (mPV) 1 was defined in the 2016 World Health Organisation classification of MPN 2 as a new JAK2V617F-positive entity with a phenotypic presentation mimicking ET (apparently isolated thrombocytosis) but associated to endogenous erythroid colony formation (EEC) as found in PV or histological findings of PV (initially described as latent or inapparent PV). Classical Philadelphia-negative myeloproliferative neoplasms (MPN) are characterized by the presence of driver mutations ( JAK2, CALR or MPL) and comprise polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF).
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