TY - JOUR
T1 - Masked polycythemia Vera (mPV)
T2 - Results of an international study
AU - Barbui, Tiziano
AU - Thiele, Jürgen
AU - Gisslinger, Heinz
AU - Finazzi, Guido
AU - Carobbio, Alessandra
AU - Rumi, Elisa
AU - Luigia Randi, Maria
AU - Betozzi, Irene
AU - Vannucchi, Alessandro M.
AU - Pieri, Lisa
AU - Carrai, Valentina
AU - Gisslinger, Bettina
AU - Müllauer, Leonhard
AU - Ruggeri, Marco
AU - Rambaldi, Alessandro
AU - Tefferi, Ayalew
PY - 2014/1
Y1 - 2014/1
N2 - 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 <16.5 g/dL in females (range 15.0-16.4). This cohort operationally referred to as masked PV (mPV) was compared with 257 patients with overt PV and displayed male predominance, a more frequent history of arterial thrombosis and thrombocytosis. Incidence of thrombosis was similar between the two groups but mPV displayed significantly higher rates of progression to myelofibrosis and acute leukemia and inferior survival. In multivariable analysis mPV diagnosis was an independent predictor of poor survival along with age >65 years and leukocyte count >10 × 109/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.
AB - 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 <16.5 g/dL in females (range 15.0-16.4). This cohort operationally referred to as masked PV (mPV) was compared with 257 patients with overt PV and displayed male predominance, a more frequent history of arterial thrombosis and thrombocytosis. Incidence of thrombosis was similar between the two groups but mPV displayed significantly higher rates of progression to myelofibrosis and acute leukemia and inferior survival. In multivariable analysis mPV diagnosis was an independent predictor of poor survival along with age >65 years and leukocyte count >10 × 109/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.
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U2 - 10.1002/ajh.23585
DO - 10.1002/ajh.23585
M3 - Article
C2 - 23996471
AN - SCOPUS:84892882972
SN - 0361-8609
VL - 89
SP - 52
EP - 54
JO - American journal of hematology
JF - American journal of hematology
IS - 1
ER -