TY - JOUR
T1 - Polycythemia vera-associated complications
T2 - Pathogenesis, clinical manifestations, and effects on outcomes
AU - Cuthbert, Danielle
AU - Stein, Brady Lee
N1 - Publisher Copyright:
© 2019 Cuthbert and Stein.
PY - 2019
Y1 - 2019
N2 - Polycythemia vera is a Philadelphia-negative chronic myeloproliferative neo-plasm, characterized by erythrocytosis, which is unique, compared to essential thrombocy-tosis and primary myelofibrosis. Though longevity can usually be expected, vascular morbidity is associated with this condition, as well as a propensity to evolve into myelofi-brosis (post-PV MF) and acute myeloid leukemia. In addition, patients can have a pro-nounced symptom burden. Herein, contributors to the symptomatic burden, as well as the thrombotic and transformative tendencies are reviewed. From a symptom perspective, some are explained by cytokine release, others by microvascular complications, whereas certain symptoms can herald disease evolution. Thrombosis has multifactorial contributors, including but not limited to gender, and inflammatory stress; investigators have recently hypothe-sized that microparticles and Neutrophil Extracellular Trap Formations may add to thrombotic burden. Finally, we examine the progression to post-PV MF as well as leukemic transformation, highlighting well-established risk factors including age and leukocytosis, certain treatments, and the presence of “non-driver” mutations.
AB - Polycythemia vera is a Philadelphia-negative chronic myeloproliferative neo-plasm, characterized by erythrocytosis, which is unique, compared to essential thrombocy-tosis and primary myelofibrosis. Though longevity can usually be expected, vascular morbidity is associated with this condition, as well as a propensity to evolve into myelofi-brosis (post-PV MF) and acute myeloid leukemia. In addition, patients can have a pro-nounced symptom burden. Herein, contributors to the symptomatic burden, as well as the thrombotic and transformative tendencies are reviewed. From a symptom perspective, some are explained by cytokine release, others by microvascular complications, whereas certain symptoms can herald disease evolution. Thrombosis has multifactorial contributors, including but not limited to gender, and inflammatory stress; investigators have recently hypothe-sized that microparticles and Neutrophil Extracellular Trap Formations may add to thrombotic burden. Finally, we examine the progression to post-PV MF as well as leukemic transformation, highlighting well-established risk factors including age and leukocytosis, certain treatments, and the presence of “non-driver” mutations.
KW - Leukemic transformation
KW - Myelofibrosis
KW - Polycythemia vera
KW - Symptomatic burden
KW - Thrombosis
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U2 - 10.2147/JBM.S189922
DO - 10.2147/JBM.S189922
M3 - Review article
C2 - 31695542
AN - SCOPUS:85074191905
SN - 1179-2736
VL - 10
SP - 359
EP - 371
JO - Journal of Blood Medicine
JF - Journal of Blood Medicine
ER -