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Recently reported mature survival data have confirmed the favorable prognosis in polycythemia vera PVwith an estimated median survival of 24 years, in patients younger than age 60 years old.
Currently available drugs for PV have not been shown to prolong survival or alter the natural history of the disease and are instead indicated primarily for prevention of thrombosis.
Unfortunately, study endpoints that are being utilized in currently ongoing clinical trials in PV do not necessarily target clinically or biologically relevant outcomes, such as thrombosis, survival, or morphologic remission, and are instead focused on components of disease palliation. In addition, it is reasonable to consider JAK2 inhibitor therapy, in the presence of protracted pruritus or markedly enlarged splenomegaly shown to be refractory to the aforementioned drugs.
In addition, bone marrow morphologic assessment is encouraged, in order to distinguish PV from JAK2 -mutated ET 567 and obtain cytogenetic information, which has recently been shown to be prognostically relevant 89 Clinical features in PV include mild-to-moderate degree of splenomegaly, mild-to-moderate degree of constitutional symptoms, including fatigue and pruritus, symptoms of hyperviscosity, leukocytosis, thrombocytosis, microvascular symptoms e.
Current treatment in PV has not affected the natural history of the disease in regards to overall, leukemia-free or myelofibrosis-free survival, but thrombosis-free survival has been positively affected by treatment with phlebotomy 12aspirin 13 and cytoreductive drugs In the latter regard, the most popular and evidence-supported cytoreductive agent is hydroxyurea, while busulfan has been effectively and safely utilized for an even longer period 11 In the current review, we provide a risk-adapted treatment algorithm in PV, including critical assessment of the currently available cytoreductive agents.
Survival in PV is inferior to that of ET but superior to that of PMF, with estimated medians of 14, 20, and 6 years, respectively; 15 the corresponding figures for patients younger than age 60 years are 24, 33, and 15 years Life-expectancy in all three MPN is significantly worse than that of the age- and sex-matched general population These observations are similar to those from a large population-based study of 9, patients The major life-threatening complications in PV are leukemic transformation, fibrotic progression and thrombosis, with incidence ranges of 5.
Some of these mutations, in particular ASXL1, SRSF2or IDH2have been shown to adversely impact overall and transformation-free survival; median survival of patients with and without adverse mutations was 7. After a median follow-up of 2. The potential contribution of increased leukocyte count to thrombosis in PV was also highlighted in the context of the CYTO-PV study 26 and recurrent thrombosis, especially in patients younger than age 60 years 27 In the IWG-MRT study, arterial and venous thromboses were the main risk factors for recurrent arterial or venous vascular events, respectively A more recent study has suggested that arterial hypertension might be a significant risk factor for thrombosis, even in low-risk patients Another study suggested that PV patients with bone marrow fibrosis might be at a lower risk for thrombosis On the basis of the above, we consider PV patients with thrombosis history to be at a significantly higher risk for recurrent thrombosis; in this regard, it is therapeutically relevant to distinguish patients with arterial vs venous thrombosis history Fig.
In addition, although not included in our current risk stratification scheme, we take the presence of hypertension and leukocytosis into consideration, when deciding treatment in certain circumstances Fig. Prior to the introduction of phlebotomy as a treatment modality in PV, reported median survivals for untreated PB were less than 2 years and attributed to excess death from thrombotic complications Controlled studies have also confirmed the additional anti-thrombotic value of low-dose aspirin in PV, among all risk categories In addition, the emerging data from laboratory studies and clinical observations suggest that the increased platelet turnover in MPN results in suboptimal hour suppression of thromboxane-A2 synthesis by once-daily dosing; 3233 therefore, we consider twice-daily dosing in low-risk patients whose microvascular symptoms are not adequately controlled with once-daily dosing or who are at high-risk for arterial thrombosis, including those with cardiovascular risk factors especially hypertension and leukocytosis Fig.
Most recently, two studies have re-visited the issue of the frequency of phlebotomy and thrombosis risk in PV, based on older polycythemia vera study group data that suggested increased risk of thrombosis in the first 3 years of treatment, in patients treated with phlebotomy alone 34 In the first observational study 35the authors showed an association between requiring 3 or more phlebotomies per year and increased risk of thrombosis, despite concomitant treatment with hydroxyurea.
This observation was not confirmed by the more robust second study that utilized data from a controlled study and implicated uncontrolled hematocrit level instead of frequency of phlebotomy as the culprit; 34 furthermore, there is additional evidence that suggests the contribution of leukocytosis to the increased risk of thrombosis in patients with inadequate control of hematocrit Our current recommendations on the management of high-risk PV are based on both controlled and large retrospective and single arm prospective studies Fig.
The PVSG conducted the first controlled study in PV — that compared treatment with phlebotomy alone or in combination with either oral chlorambucil or intravenous radioactive phosphorus P The results revealed accelerated leukemic transformation and shortening of survival in patients receiving chlorambucil of P32 36 The above-outlined observations on hydroxyurea treatment for PV were further supported by several other uncontrolled studies, including a PVSG study where the drug was associated with lower incidences of thrombosis, compared to a historical cohort treated with phlebotomy alone 6.
Several other uncontrolled studies have since confirmed the lack of association between hydroxyurea treatment and leukemic transformation with reported incidence range of 1—5.Once production of your article has started, you can track the status of your article via Track Your Accepted Article. This respected international journal, Blood Reviewsis a vital information resource, bringing together appraisals of clinical practice, and research from recognized experts.
Specially commissioned peer reviewed articles from leading researchers and practitioners guarantee truly global coverage of all Specially commissioned peer reviewed articles from leading researchers and practitioners guarantee truly global coverage of all the sub-specialties of hematology.
Blood Reviews publishes review articles covering the spectrum of clinical and laboratory haematological practice and research. Although most reviews are invited, the editors welcome suggestions from potential authors.
They should first write a brief outline of an intended review and send this to Dr Drew Provan a. All articles are subject to peer review. Author queries can also be sent to the Blood Reviews editorial office BloodReviews elsevier.
Benefits to authors We also provide many author benefits, such as free PDFs, a liberal copyright policy, special discounts on Elsevier publications and much more. Please click here for more information on our author services. Please see our Guide for Authors for information on article submission.
If you require any further information or help, please visit our Support Center. Search in:.
Polycythemia vera treatment algorithm 2018
Home Journals Blood Reviews. ISSN: X. Blood Reviews. Co Editor-in-Chiefs: H. View Editorial Board. CiteScore: 6. CiteScore values are based on citation counts in a given year e. Impact Factor: 6. Submit Your Paper. Supports Open Access. View Articles. Track Your Paper Check submitted paper Check the status of your submitted manuscript in the submission system Track accepted paper Once production of your article has started, you can track the status of your article via Track Your Accepted Article.
Order Journal Institutional subscription Personal subscription. Sample Issue. Journal Metrics CiteScore : 6. View More on Journal Insights.Peter E. Members should register with Wiley to receive full journal access and email alerts about new publication issues and articles.
PBC publishes manuscripts describing basic and clinical investigations of blood disorders and malignant diseases of childhood, including diagnosis, treatment, epidemiology, etiology, biology, and molecular and clinical genetics of these diseases as they affect children, adolescents, and young adults. The journal also includes the section "On Children, Blood, and Cancer" which features personal perspectives on the experiences of children with cancer or blood disorders, or of caring for these children.
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Skip to main content. Features include dynamic figures, tables, and references early view articles as they publish the ability to browse content before downloading an issue the ability to share articles via e-mail and social media access to your institutional or personal subscription.Blood - Impact Factor. The Impact Factor of Blood is The Impact Factor IF or Journal Impact Factor JIF of an academic journal is a scientometric index that reflects the yearly average number of citations that recent articles published in a given journal received.
It is frequently used as a proxy for the relative importance of a journal within its field; journals with higher impact factors are often deemed to be more important than those with lower ones.
The Impact Factor measures the average number of citations received in a particular year by papers published in the journal during the two preceding years Note that impact factors are reported in ; they cannot be calculated until all of the publications have been processed by the indexing agency. In addition to the 2-year Impact Factor, the 3-year Impact Factor and 5-year Impact Factor can provide further insights into the impact of Blood. Impact Factor Trend Prediction System provides an open, transparent, and straightforward platform to help academic researchers Predict future journal impact and performance through the wisdom of crowds.
Impact Factor Trend Prediction System displays the exact community-driven Data without secret algorithms, hidden factors, or systematic delay.
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Blood - ISSN. An ISSN is an 8-digit code used to identify newspapers, journals, magazines and periodicals of all kinds and on all media—print and electronic. Blood - Subscription non-OA Journal. Blood is a Subscription-based non-OA Journal. Publishers own the rights to the articles in their journals. Anyone who wants to read the articles should pay by individual or institution to access the articles. Anyone who wants to use the articles in any way must obtain permission from the publishers.
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Blood is a peer-reviewed scientific journal. Blood - Journal Metrics. It is impossible to get a true picture of impact using a single metric alone, so a basket of metrics is needed to support informed decisions. Ranking by Impact Factor. Ranking by Popularity. Academic Accelerator Impact Factor Database. Impact Factor Database Search Engine. Academic Accelerator Impact Factor Blood.