Evidence and expertise in the management of polycythemia vera and essential thrombocythemia

Finazzi, Guido; Barbui, Tiziano

doi:10.1038/leu.2008.177

Cited by 92 publications

(56 citation statements)

References 80 publications

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“…Regarding Hct target, the need of keeping a value<0.45, as recommended by guidelines [18,29,35] has been recently questioned by the ECLAP study [30]. Unfortu- nately, few patients (22% only) in our survey were actually maintained at an Hct value<0.45, this suggesting the difficulties for patients and/or physicians to comply with published guidelines, in agreement to other reports [30,36].…”

Section: Discussionsupporting

confidence: 84%

“…Nevertheless, the high thrombotic risk according to Finazzi et al [28,29] confirmed its negative prognostic impact in terms of overall and thrombosis-free survival in both univariate and multivariate analysis.…”

Section: Discussionmentioning

confidence: 86%

“…These parameters were used to define the thrombotic risk according to the recent definition by Finazzi and Barbui based on ECLAP data [28,29] (32,33) c Percent of chemotherapy-treated patients; some patients received more than one treatment for platelet counts were 400×10 9 /l, corresponding to the normal upper range, and 600×10 9 /l, the thrombocytosis threshold previously used by WHO guidelines for the diagnosis of essential thrombocythemia [31]. A leukocyte cutoff value of 15×10 9 /l was chosen, since it has been previously reported to be prognostically relevant in terms of thrombosis-free survival [32].…”

Section: Methodsmentioning

confidence: 99%

“…Lowdose acetyl-salicylic acid (ASA) has been recommended too, unless contraindicated, in order to reduce thrombotic risk and mortality; conversely cytoreductive therapy is recommended only in the presence of poor tolerance to phlebotomy, symptomatic or progressive splenomegaly or evidence of high thrombotic risk or disease progression [18,[26][27][28][29].…”

Section: Introductionmentioning

confidence: 99%

“…Indeed, a recent European survey on a very large casistics (ECLAP study) questioned the actual role of a low (<0.45) Hct value in reducing the thrombotic risk [29,30]. Moreover, the same study evidenced that even in the setting of a clinical study, about 60% of patients did not reach or maintain the recommended Hct value [30].…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

A retrospective study on 226 polycythemia vera patients: impact of median hematocrit value on clinical outcomes and survival improvement with anti-thrombotic prophylaxis and non-alkylating drugs

et al. 2010

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Section: Discussionsupporting

confidence: 84%

Section: Discussionmentioning

confidence: 86%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

A retrospective study on 226 polycythemia vera patients: impact of median hematocrit value on clinical outcomes and survival improvement with anti-thrombotic prophylaxis and non-alkylating drugs

et al. 2010

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ASH 2009 meeting report—Top 10 clinically oriented abstracts in myeloproliferative neoplasms

Tefferi

2010

American J Hematol

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Annual Clinical Updates in Hematological Malignancies: A Continuing Medical Education Series: Polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk‐stratification, and management

Tefferi

2011

American J Hematol

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Disease overview: Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms primarily characterized by erythrocytosis and thrombocytosis, respectively. Other disease features include leukocytosis, splenomegaly, thrombohemorrhagic complications, vasomotor disturbances, pruritus, and a small risk of disease progression into acute leukemia or myelofibrosis. Diagnosis: Diagnosis is based on JAK2 mutation status (PV and ET), serum erythropoietin (Epo) level (PV), and bone marrow histopathology (ET). The presence of a JAK2 mutation and subnormal serum Epo level confirm a diagnosis of PV. Differential diagnosis in ET should include chronic myelogenous leukemia and prefibrotic myelofibrosis. Risk stratification: Current risk stratification in PV and ET is designed to estimate the likelihood of thrombotic complications: high‐risk‐age >60 years or presence of thrombosis history; low‐risk‐absence of both of these two risk factors. Presence of extreme thrombocytosis (platelet count >1,000 × 109/L) might be associated with acquired von Willebrand syndrome (AvWS) and, therefore, risk of bleeding. Risk factors for shortened survival in both PV and ET include age >60 years, leukocytosis, history of thrombosis, and anemia. Risk‐adapted therapy: Survival is near‐normal in ET and reasonably long in PV. The 10‐year risk of leukemic/fibrotic transformation is <1%/1% in ET and <5%/10% in PV. In contrast, the risk of thrombosis exceeds 20%. The main goal of therapy is therefore to prevent thrombohemorrhagic complications and this is effectively and safely accomplished by the use of low‐dose aspirin (PV and ET), phlebotomy (PV), and hydroxyurea (high risk PV and ET). Treatment with busulfan or interferon‐α is usually effective in hydroxyurea failures. Am. J. Hematol. 86:293–301, 2011. © 2011 Wiley‐Liss, Inc.

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Evidence and expertise in the management of polycythemia vera and essential thrombocythemia

Cited by 92 publications

References 80 publications

A retrospective study on 226 polycythemia vera patients: impact of median hematocrit value on clinical outcomes and survival improvement with anti-thrombotic prophylaxis and non-alkylating drugs

A retrospective study on 226 polycythemia vera patients: impact of median hematocrit value on clinical outcomes and survival improvement with anti-thrombotic prophylaxis and non-alkylating drugs

ASH 2009 meeting report—Top 10 clinically oriented abstracts in myeloproliferative neoplasms

Annual Clinical Updates in Hematological Malignancies: A Continuing Medical Education Series: Polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk‐stratification, and management

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