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Masked polycythemia Vera (mPV): Results of an international study

2013; Wiley; Volume: 89; Issue: 1 Linguagem: Inglês

10.1002/ajh.23585

1096-8652

Tiziano Barbui, Jürgen Thiele, Heinz Gisslinger, Guido Finazzi, Alessandra Carobbio, Elisa Rumi, Maria Luigia Randi, Irene Betozzi, Alessandro M. Vannucchi, Lisa Pieri, Valentina Carrai, Bettina Gisslinger, Leonhard Müllauer, Marco Ruggeri, Alessandro Rambaldi, Ayalew Tefferi,

Kruppel-like factors research

We examined the baseline features and clinical outcomes of 140 patients presenting with JAK2V617F positivity and a bone marrow morphology conforming with WHO criteria of polycythemia vera (PV), but a hemoglobin level of <18.5 g/dL in males (range 16.0-18.4) and 65 years and leukocyte count >10 × 10(9) /L. Our data suggest that mPV is a heterogeneous myeloproliferative neoplasia and not necessarily an early/ pre-polycythemic form of classical PV that at onset in a small fraction of patients clinically may mimic essential thrombocythemia. On the other hand, the majority mPV may have a longer prodrome of undiagnosed PV or a disease biology akin to primary myelofibrosis-post PV myelofibrosis that could explain the worsening of outcome in comparison to overt/classical manifestations.