Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia

Passamonti, Francesco; Rumi, Elisa; Pungolino, Ester; Malabarba, Lucia; Bertazzoni, Paola; Valentini, Marina; Orlandi, Ester; Arcaini, Luca; Brusamolino, Ercole; Pascutto, Cristiana; Cazzola, Mario; Morra, Enrica; Lazzarino, Mario

doi:10.1016/j.amjmed.2004.06.032

Cited by 409 publications

(366 citation statements)

References 23 publications

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“…These diseases are usually complicated by thrombosis, with a rate of major thrombosis as high as about 50% [1][2][3][4]. Advanced age and a prior history of thrombosis are the two most important risk factors for vascular complications, while hyper-cholesterolemia, hypertension, smoking, and diabetes have been recognized as predictors of thrombosis [1][2][3][4]. In the last few years, new information on the pathogenesis of thrombosis in MPNs became available, including the role of leukocyte activation and interaction with platelets as well as the presence of JAK2 V617F mutation [5,6].…”

Section: Introductionmentioning

confidence: 99%

Correlation between leukocytosis and thrombosis in Philadelphia-negative chronic myeloproliferative neoplasms

et al. 2009

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International audienceThe evidence that leukocytes may contribute to the pathogenesis of thrombosis in Chronic Myeloproliferative Neoplasms is increasing but not definitive. To further enforces whether an increased leukocyte count is associated with thrombosis and whether this effect can be modulated by cytoreductive therapy, we analyzed the clinical course of 187 patients with Polycythemia Vera (PV) and Essential Thrombocythemia (ET) followed at two Italian Institutions over a period of 7 years. The association was measured at diagnosis or before thrombotic events: a multivariable analysis was carried out using data at baseline and time-dependent covariates. We found that white blood cells (WBC) count above 9.5 × 10/L at diagnosis (baseline analysis) was associated with thrombosis during the follow-up (Hazard Ratio [HR] of 1.8, 0.03). At the time-dependent analysis, therapy with hydroxyurea (HU), lowering by 35% the baseline WBC level, reduced such strength of association giving a HR of 1.3 ( value non significant). We found a trend between WBC level and thrombosis in untreated low-risk patients (RR of 1.9, 95% CI 0.9 to 3.1); in high-risk patients treated with HU this correlation was clearly lost (RR 1.1, 95% CI 0.2 to 2.7). Finally, we could not identify the presence of as a risk factor for thrombosis. Properly designed prospective studies should corroborate such results

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Section: Introductionmentioning

confidence: 99%

Correlation between leukocytosis and thrombosis in Philadelphia-negative chronic myeloproliferative neoplasms

et al. 2009

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“…In PV, less than 20 % of the patients may present with a certain degree of myelofibrosis (MF) at the time of diagnosis and transformation from PV to MF has been reported to occur at a ratio about 5-15 % after 15-20 years of follow-up [2,3]. Since the incidence of MF in first 10 years is low and it is usually manifested in patients with a long duration of disease, the occurrence of MF is rather early in our patient and may suggest an aggressive subgroup [4].…”

Section: Discussionmentioning

confidence: 99%

“…Post-polycythemia vera myelofibrosis (post-PV MF) is a long term clinical outcome in around 10 % of patients with polycythemia vera (PV) [1,2]. No clear risk factors have been identified for such a transformation.…”

Section: Introductionmentioning

confidence: 99%

A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis

Değertekin

Akyürek

Yağcı

2012

Indian J Hematol Blood Transfus

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Polycythemia vera is a clonal proliferative disorder of the bone marrow that could possibly evolve into myelofibrosis in its natural course. Progression to myelofibrosis is usually a late stage complication and presents clinically with refractory cytopenias and extramedullary hematopoiesis (EMH). EMH can occur in any tissue during the course of post-polycythemic myelofibrosis. However, skin and cardiac involvements seems to be very rare. We present a 56-year-old woman with post-polycythemic myelofibrosis refractory to treatment, developing EMH after splenectomy in various organs, exceptionally the skin and the heart. Along with the case, the clinical presentations, treatment options, prognostic significance of EMH and the role of cytogenetics is discussed in the light of the literature.

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“…No substantial differences between life expectancy of 247 patients with ET, according to the PVSG criteria and that of a control population were found by Rozman et al [76,81]. Nevertheless, the common feeling that ET is a rather benign MPD, affecting more the quality of life than the overall life expectancy, has been challenged by recent publications.…”

Section: Prognosismentioning

confidence: 96%

“…A comparison, although retrospective, between PI and HU has been recently assessed. After a median follow-up of 104 months in a cohort study of 155 ET patients, a significantly lower transformation rate was observed in PItreated patients [76].…”

Section: Side Effects and Mutagenic Potentialmentioning

confidence: 96%

Essential Thrombocythemia

Encyclopedia of Diagnostic Imaging

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Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia

Cited by 409 publications

References 23 publications

Correlation between leukocytosis and thrombosis in Philadelphia-negative chronic myeloproliferative neoplasms

Correlation between leukocytosis and thrombosis in Philadelphia-negative chronic myeloproliferative neoplasms

A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis

Essential Thrombocythemia

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