Vascular and Neoplastic Risk in a Large Cohort of Patients With Polycythemia Vera

Marchioli, Roberto; Finazzi, Guido; Landolfi, Raffaele; Kutti, Jack; Gisslinger, Heinz; Patrono, Carlo; Marilus, Raphael; Villegas, A; Tognoni, Gianni; Barbui, Tiziano

doi:10.1200/jco.2005.07.062

Cited by 643 publications

(661 citation statements)

References 19 publications

Supporting

Mentioning

591

Contrasting

Unclassified

Order By: Relevance

“…Low-dose ASA might have also contributed to the relatively low incidence of major thrombosis (18%), in the lower range of literature data [6,27], without increasing hemorrhagic complications compared to published observations [33]. 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: 83%

“…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%

See 1 more Smart Citation

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

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 83%

Section: Methodsmentioning

confidence: 99%

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

View full text Add to dashboard Cite

“…43 The largest prospective evaluation of risk factors for survival, incidence of thrombosis and hematological transformation is the ECLAP observational cohort study. 44 Patients younger than 65 years in the ECLAP Study had the lowest rate of thrombosis (2.5% patients per year), while with age older than 65 years and a positive history of thrombosis, the corresponding rate ranged from 6 to 10% patients per year. Of note were a consistent association between age 465 years and risk of leukemia and between duration of disease with risk of overt myelofibrosis.…”

Section: What To Do In Young Patients With Thrombosismentioning

confidence: 89%

How to manage children and young adults with myeloproliferative neoplasms

Barbui

2012

Leukemia

View full text Add to dashboard Cite

On the basis of my personal clinical and research experience and validated by the current literature, my approach to the management of pediatric (age o18 years) and young patients (age o40 years) with classic myeloproliferative neoplasms is presented by focusing on diagnosis, patient communication, risk stratification and therapy. The WHO-2008 diagnostic criteria are recommended, even though in children suspected with essential thrombocythemia (ET), a specific set of diagnostic features may be required. Patient communication includes information on natural history, genetic abnormalities and counseling in all women of child-bearing age. The main challenge in children and young adults with ET and polycythemia vera (PV) is to avoid recurrence of major thrombosis by selecting those patients who ultimately can benefit from cytotoxic and antithrombotic therapy without increasing the incidence of drug-induced side effects. In asymptomatic low-risk patients no therapy is prescribed while in high-risk low-dose aspirin, hydroxyurea and interferon-alpha are my first line drugs. My first decision when considering treatment of a young patient with primary myelofibrosis (PMF) or post-PV or post ET-myelofibrosis, is whether he/she qualifies for bone marrow allotransplantation. In the remaining young PMF patients palliative therapy or experimental drugs are considered.Leukemia ( Keywords: myeloproliferative neoplasms; treatment; young people INTRODUCTION Polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) are currently classified among the bcr/abl-negative, 'classic' myeloproliferative neoplasms (MPNs) and represent a stem cell-derived clonal myeloproliferation. Hematopoietic progenitors derived from patients with MPNs are hypersensitive to the stimulation of physiological growth factors such as thrombopoietin or erythropoietin (EPO). 1 Since 2005, the pathophysiology of these diseases has advanced considerably with the discovery of an acquired mutation of JAK2 V617F in a vast majority of PV patients and in almost half of those with ET and PMF. 2 The mutation, located within the negative regulatory pseudokinase, or Janus homology 2 domain, causes cytokine-independent activation of several biochemical pathways implicated in EPO receptor signaling. Subsequently, mutations in MPL were reported in B4% of patients with ET or PMF. The significance of JAK2 and MPL mutations for the evolution of the MPNs and their relative roles in determining disease phenotype as well as progression to myelofibrosis and leukemic transformation are unclear at present.In this context, it should be underscored that most of the information in terms of diagnosis, prognosis and therapy focuses on the 'average' MPN patients of whom median age ranges between 60 and 65 years. On the other hand, less consistent data are available in the groups of MPN patients who are presenting below this median age such as pediatric and young adults (20% of MPN patients). This issue is now becoming more and more important, because with the ad...

show abstract

“…According to knowledge from analyses done in the past era of the PVSG classification [7], older age ( 60 years) and a history of thrombosis are considered as standard risk factors for thrombosis and guide current treatment decisions [11]. Leukocytosis at diagnosis and the JAK2V617F mutation are suspected as novel disease-associated risk factors for vascular complications [12][13][14].…”

Section: Discussionmentioning

confidence: 99%

Leukocytosis as an important risk factor for arterial thrombosis in WHO‐defined early/prefibrotic myelofibrosis: An international study of 264 patients

et al. 2012

Self Cite

View full text Add to dashboard Cite

We aimed to determine risk factors for thrombotic events in early/prefibrotic myelofibrosis diagnosed according to the World Health Organization criteria. Multivariate Cox regression analysis was calculated on a total number of 264 patients derived from an international database. After a median follow‐up of 6.28 years, 42 (15.9%) patients experienced arterial (n = 31) or venous thrombosis (n = 11). A higher leukocyte count correlated with an increased risk for total thrombosis and in particular, with an increased risk for arterial thrombosis (P = 0.005, HR 1.15 and P = 0.047, HR 1.12, respectively). A platelet count above 870 × 109/L was associated with a lower risk for total thrombosis and also for venous thrombosis (P = 0.022, HR 0.44 and P = 0.027, HR 0.19). Moreover, a lower hemoglobin level was associated with an increased risk for venous thrombosis (P = 0.007, HR 0.59). Our data indicate that leukocytosis is a prominent risk factor for thrombosis in early/prefibrotic MF. Am. J. Hematol. 87:669–672, 2012. © 2012 Wiley Periodicals, Inc.

show abstract

Vascular and Neoplastic Risk in a Large Cohort of Patients With Polycythemia Vera

Cited by 643 publications

References 19 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

How to manage children and young adults with myeloproliferative neoplasms

Leukocytosis as an important risk factor for arterial thrombosis in WHO‐defined early/prefibrotic myelofibrosis: An international study of 264 patients

Contact Info

Product

Resources

About