Long-term follow-up of essential thrombocythemia in young adults: treatment strategies, major thrombotic complications and pregnancy outcomes. A study of 76 patients

Palandri, Francesca; Polverelli, Nicola; Ottaviani, Emanuela; Castagnetti, Fausto; Baccarani, Michele; Vianelli, Nicola

doi:10.3324/haematol.2009.019190

Cited by 24 publications

(28 citation statements)

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“…This cohort of patients represents one of the first elderly ET populations studied to date. Very elderly patients seemed to constitute a separate category characterized by a high thrombotic risk (30% at 10 years), which was higher than that observed in patients aged 60-74 (21AE9%), and was also substantially greater than that reported in our general (12AE6%) (Palandri et al, 2009a) and young (7AE5%) (Palandri et al, 2010) series. The negative impact of ageing on thrombotic risk is also acknowledged in the healthy population (Engbers et al, 2010); additionally, the great majority of evaluable patients (81%) carried the JAK2 V617F mutation, which may have contributed to the increased vascular risk (Vannucchi et al, 2007).…”

contrasting

confidence: 47%

Very elderly patients with essential thrombocythaemia: are they a separate category? A monocentric study on 118 patients older than 75 years

et al. 2011

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contrasting

confidence: 47%

Very elderly patients with essential thrombocythaemia: are they a separate category? A monocentric study on 118 patients older than 75 years

et al. 2011

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“…45,46 Antithrombotic drugs and the use of cytoreductive therapy to control the myeloproliferative process are the mainstay of secondary prophylaxis.…”

Section: What To Do In Young Patients With Thrombosismentioning

confidence: 99%

How to manage children and young adults with myeloproliferative neoplasms

Barbui

2012

Leukemia

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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...

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“…Podawanie leku należy przerwać 2 tygodnie przed planowanym porodem, a w okresach okołoporodowym i połogu zastosować heparyny drobnocząsteczkowe. W okresie połogu należy stosunkowo często kontrolować liczbę PLT, ponieważ w krótkim czasie po porodzie ich liczba powraca do wartości sprzed ciąży, a często nawet ją przekracza [38,39].…”

Section: Nadpłytkowość Samoistnaunclassified

Problemy hematologiczne w okresie ciąży

Kopeć

2017

Hematologia

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Long-term follow-up of essential thrombocythemia in young adults: treatment strategies, major thrombotic complications and pregnancy outcomes. A study of 76 patients

Cited by 24 publications

References 11 publications

Very elderly patients with essential thrombocythaemia: are they a separate category? A monocentric study on 118 patients older than 75 years

Very elderly patients with essential thrombocythaemia: are they a separate category? A monocentric study on 118 patients older than 75 years

How to manage children and young adults with myeloproliferative neoplasms

Problemy hematologiczne w okresie ciąży

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