Goal of Therapy and Monitoring the Response in Polycythemia Vera and Essential Thrombocythemia

  • Jean-Jacques Kiladjian
Part of the Hematologic Malignancies book series (HEMATOLOGIC)


Polycythemia vera (PV) and essential thrombocythemia (ET) are the most indolent Philadelphia-negative myeloproliferative neoplasms (MPN). In fact, several studies have shown that life expectancy in ET is almost similar to that of the general age- and sex-matched population, but life expectancy of PV patients is however significantly altered, especially after the first decade of follow-up (Passamonti et al. 2004; Cervantes et al. 2008). Evolution of PV and ET is characterized by a short-term risk of vascular complications (thrombosis and hemorrhages) and a longer-term risk of evolution to myelofibrosis (MF), myelodysplastic syndromes (MDS), or acute myeloid leukemia (AML). However, in a recent retrospective study from the Swedish Cancer Registry that included 4,389 and 2,559 patients with a diagnosis of PV and ET, respectively, a significant overall excess mortality compared to reference population was found (Bjorkholm et al. 2011). Contrary to high-risk MF where treatment aims to prolong survival that may require therapies with high risks of complications, current therapeutic strategies in PV and ET are based on the risk of thrombosis, aiming to reduce mortality and morbidity due to vascular events (Barbui et al. 2011a).


Acute Myeloid Leukemia Vascular Complication Polycythemia Vera Interferon Alpha Essential Thrombocythemia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  1. 1.Clinical Investigations CenterSaint-Louis Hospital and Paris Diderot UniversityParisFrance

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