Polycythæmia Vera. Course and Prognosis<sup>1</sup>

Videbaek, A

doi:10.1111/j.0954-6820.1950.tb10111.x

Cited by 123 publications

(14 citation statements)

References 5 publications

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“…This question led us to explore the role of the unique host as a modifier of disease pathogenesis. We had previously observed gender differences in disease distribution, including an overrepresentation of women among those with ET and PV, and an underrepresentation of women among those with PMF [1]; also, we found that similar to historical data [2], PV was manifest at a younger age in women compared to men in our cohort. Therefore, we identified gender as a potential host modifier of disease pathogenesis and evaluated whether there were also differences in the JAK2 V617F allele burden between men and women.…”

Section: Introductionsupporting

confidence: 85%

“…Historical differences in the experience of extramedullary hematopoiesis have been reported. For example, it has been suggested that splenomegaly more commonly complicated the course in women with PV, but survival was better compared to men [2]. Historical literature has also suggested that women with myelofibrosis have a longer duration of illness leading up to splenectomy, a lower complication rate, and a higher survival associated with this procedure compared to men [4].…”

Section: Introductionmentioning

confidence: 99%

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Gender and Vascular Complications in the JAK2 V617F-Positive Myeloproliferative Neoplasms

et al. 2011

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We previously found that gender influenced the JAK2 V617F allele burden, but it is unknown whether this gender difference in molecular epidemiology influences complications in the myeloproliferative neoplasms (MPNs). Historically, vascular complications represented the most common cause of mortality in polycythemia vera and essential thrombocytosis and contributed to morbidity in primary myelofibrosis. To determine the influence of gender on vascular complications, we retrospectively analyzed associations between gender and vascular complications. Despite their younger age, less prevalent dyslipidemia or smoking history, lower white blood counts, and lower JAK2 V617F allele burden, women had higher rates of abdominal venous thrombosis and comparable rates of all vascular complications. Vascular risk is currently not easily stratified by MPN-disease burden or traditional risk factors. Our analysis contributes to growing literature emphasizing gender differences in the MPN and further supports the important impact of individual and host variation on MPN clinical manifestations, and especially vascular risk.

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Section: Introductionsupporting

confidence: 85%

Section: Introductionmentioning

confidence: 99%

Gender and Vascular Complications in the JAK2 V617F-Positive Myeloproliferative Neoplasms

et al. 2011

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“…The annual genderspecific incidence rates were 2.4 per 10 5 male inhabitants compared to 3.08 per 10 5 female inhabitants. 13 Confirming historical data, 6 we have observed that polycythemia vera is manifested at a younger age in women than in men. We have also observed differences in disease distribution, including an over-representation of women among those with essential thrombocytosis, and an under-representation of women among those with primary myelofibrosis.…”

Section: Introductionsupporting

confidence: 71%

Sex differences in the JAK2V617F allele burden in chronic myeloproliferative disorders

et al. 2010

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BackgroundThe JAK2 V617F allele burden is a variable measure, determined by the frequency of mitotic recombination events and the expansion of JAK2 V617F clones. Since variability in the JAK2 V617F allele burden is partly responsible for the distinct phenotypes seen in the myeloproliferative disorders, the objective of this study was to identify modifiers of the allele burden. Design and MethodsBlood samples were obtained between May 2005 and January 2009 from 272 patients with essential thrombocytosis, polycythemia vera, and myelofibrosis. The JAK2 V617F allele burden was measured by an allele-specific quantitative polymerase chain reaction using DNA from purified neutrophils. Repeated measures, on average 2 years apart, were available for 104 patients. ResultsSex, age at diagnosis, and disease duration all independently influenced the JAK2 V617F allele burden. When considering all patients with myeloproliferative disorders, women had significantly lower allele burdens than men (P=0.04). In those patients with repeated measures, the increase in allele burden per year between the first and second evaluations was significantly less in females than in males. Among those who experienced disease evolution, females were 4.5 times more likely to have evolution from essential thrombocytosis to polycythemia vera, but 0.23 times as likely to have evolution from essential thrombocytosis to myelofibrosis. ConclusionsSex is an independent factor accounting for variability in the JAK2 V617F allele burden. We speculate that lower allele burdens in females reflect a lower frequency of mitotic recombination events in females than in males, and should be considered when evaluating the relationship of allele burden to disease phenotype and also in evaluating responses to JAK2 V617F -inhibitors. Because sex may influence genotype and/or clonal expansion, underpinning the variability in JAK2 V617F allele burden, it will be important to explore factors that determine susceptibility to mitotic recombination events. disorders. Haematologica 2010;95:1090-1097. doi:10.3324/haematol.2009 This is an open-access paper. Sex differences in the JAK2

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“…119 Because clonal dominance is time-dependent, 37 the MPDs are not static disorders and their clinical phenotype is subject to change over time. As a consequence, no single diagnostic test and, frequently, no combination of diagnostic tests, is sufficient to establish the diagnosis of a particular MPD or even to distinguish the MPDs from the other benign and malignant hematologic disorders they mimic.…”

Section: Discussionmentioning

confidence: 99%

The revised World Health Organization diagnostic criteria for polycythemia vera, essential thrombocytosis, and primary myelofibrosis: an alternative proposal

Spivak

Silver

2008

Blood

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140

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Polycythæmia Vera. Course and Prognosis¹

Cited by 123 publications

References 5 publications

Gender and Vascular Complications in the JAK2 V617F-Positive Myeloproliferative Neoplasms

Gender and Vascular Complications in the JAK2 V617F-Positive Myeloproliferative Neoplasms

Sex differences in the JAK2V617F allele burden in chronic myeloproliferative disorders

The revised World Health Organization diagnostic criteria for polycythemia vera, essential thrombocytosis, and primary myelofibrosis: an alternative proposal

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Polycythæmia Vera. Course and Prognosis1

Cited by 123 publications

References 5 publications

Gender and Vascular Complications in the JAK2 V617F-Positive Myeloproliferative Neoplasms

Gender and Vascular Complications in the JAK2 V617F-Positive Myeloproliferative Neoplasms

Sex differences in the JAK2V617F allele burden in chronic myeloproliferative disorders

The revised World Health Organization diagnostic criteria for polycythemia vera, essential thrombocytosis, and primary myelofibrosis: an alternative proposal

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Polycythæmia Vera. Course and Prognosis¹