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Tiziano Barbui - One of the best experts on this subject based on the ideXlab platform.
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Polycythemia vera treatment algorithm 2018
Blood Cancer Journal, 2018Co-Authors: Ayalew Tefferi, Alessandro M Vannucchi, Tiziano BarbuiAbstract:Recently reported mature survival data have confirmed the favorable prognosis in Polycythemia vera (PV), with 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. Even more discouraging has been the lack of critical appraisal from “opinion leaders”, on the added value of newly approved drugs. Keeping these issues in mind, at present, we continue to advocate conservative management in low-risk PV (phlebotomy combined with once- or twice-daily aspirin therapy) and include cytoreductive therapy in “high-risk” patients; in the latter regard, our first, second, and third line drugs of choice are hydroxyurea, pegylated interferon-α and busulfan, respectively. 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.
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no correlation of intensity of phlebotomy regimen with risk of thrombosis in Polycythemia vera evidence from european collaboration on low dose aspirin in Polycythemia vera and cytoreductive therapy in Polycythemia vera clinical trials
Haematologica, 2017Co-Authors: Tiziano Barbui, Alessandra Carobbio, Arianna Masciulli, Alessandro Rambaldi, Arianna Ghirardi, Alessandro M VannucchiAbstract:The natural history of Polycythemia vera (PV) is marked by arterial and venous thromboembolism and evolution into myelofibrosis and/or acute myeloid leukaemia/myelodysplastic syndrome. One of the major goals of treatment is to reduce the thrombotic events which account for 40% of causes of
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white blood cell counts and thrombosis in Polycythemia vera a subanalysis of the cyto pv study
Blood, 2015Co-Authors: Tiziano Barbui, Guido Finazzi, Arianna Masciulli, Maria Rosa Marfisi, Giovanni Tognoni, Alessandro Rambaldi, Alessandro M VannucchiAbstract:To the editor: The pathogenesis of thrombosis in patients with Polycythemia vera (PV) results from a complex interplay of patient- and disease-related variables. According to age and previous thrombosis, patients are traditionally stratified as “high risk” or “low risk.” However, recently,
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in contemporary patients with Polycythemia vera rates of thrombosis and risk factors delineate a new clinical epidemiology
Blood, 2014Co-Authors: Tiziano Barbui, Maria Luigia Randi, Elisa Rumi, Alessandra Carobbio, Guido Finazzi, Francesco Rodeghiero, Alessandro Rambaldi, Heinz Gisslinger, Bettina Gisslinger, Lisa PieriAbstract:To the editor: Controlled studies in Polycythemia vera (PV) have demonstrated the value of aggressive phlebotomy, aspirin, and cytoreductive therapy in preventing thrombotic complications.[1][1][⇓][2]-[3][3] In the European Collaboration on Low-Dose Aspirin in Polycythemia vera (ECLAP)
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masked Polycythemia vera diagnosed according to who and bcsh classification
American Journal of Hematology, 2014Co-Authors: Tiziano Barbui, Maria Luigia Randi, Alessandro M Vannucchi, Elisa Rumi, Alessandra Carobbio, Guido Finazzi, Heinz Gisslinger, Jurgen Thiele, Bettina Gisslinger, Leonhard MullauerAbstract:Polycythemia vera (PV) is currently diagnosed by the World Health Organization (WHO) criteria regarding hemoglobin (HB) levels and JAK2V617F and related mutations or by the British Committee for Standards in Haematology (BCSH) guidelines predominantly based on hematocrit (HCT) values (>52% in men and >48% in women) in JAK2 mutated patients. We examined clinical features at diagnosis and outcome in 397 mutated PV patients showing a bone marrow (BM) morphology conforming with the WHO descriptions but including also cases with a HB level 65 years and white blood cell count >15 × 10(9) /L. Without these risk factors mPV patients had the same survival as overt PV suggesting that a fraction of patients with HB lower than that required for WHO diagnosis should still be considered as overt PV. This study has established the existence of mPV by two different classification systems based on either HB or HCT threshold values.
Alessandro M Vannucchi - One of the best experts on this subject based on the ideXlab platform.
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Polycythemia vera treatment algorithm 2018
Blood Cancer Journal, 2018Co-Authors: Ayalew Tefferi, Alessandro M Vannucchi, Tiziano BarbuiAbstract:Recently reported mature survival data have confirmed the favorable prognosis in Polycythemia vera (PV), with 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. Even more discouraging has been the lack of critical appraisal from “opinion leaders”, on the added value of newly approved drugs. Keeping these issues in mind, at present, we continue to advocate conservative management in low-risk PV (phlebotomy combined with once- or twice-daily aspirin therapy) and include cytoreductive therapy in “high-risk” patients; in the latter regard, our first, second, and third line drugs of choice are hydroxyurea, pegylated interferon-α and busulfan, respectively. 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.
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no correlation of intensity of phlebotomy regimen with risk of thrombosis in Polycythemia vera evidence from european collaboration on low dose aspirin in Polycythemia vera and cytoreductive therapy in Polycythemia vera clinical trials
Haematologica, 2017Co-Authors: Tiziano Barbui, Alessandra Carobbio, Arianna Masciulli, Alessandro Rambaldi, Arianna Ghirardi, Alessandro M VannucchiAbstract:The natural history of Polycythemia vera (PV) is marked by arterial and venous thromboembolism and evolution into myelofibrosis and/or acute myeloid leukaemia/myelodysplastic syndrome. One of the major goals of treatment is to reduce the thrombotic events which account for 40% of causes of
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white blood cell counts and thrombosis in Polycythemia vera a subanalysis of the cyto pv study
Blood, 2015Co-Authors: Tiziano Barbui, Guido Finazzi, Arianna Masciulli, Maria Rosa Marfisi, Giovanni Tognoni, Alessandro Rambaldi, Alessandro M VannucchiAbstract:To the editor: The pathogenesis of thrombosis in patients with Polycythemia vera (PV) results from a complex interplay of patient- and disease-related variables. According to age and previous thrombosis, patients are traditionally stratified as “high risk” or “low risk.” However, recently,
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how i treat Polycythemia vera
Blood, 2014Co-Authors: Alessandro M VannucchiAbstract:Polycythemia vera (PV) is a chronic myeloproliferative neoplasm associated with JAK2 mutations (V617F or exon 12) in almost all cases. The World Health Organization has defined the criteria for diagnosis, but it is still unclear which parameter (hemoglobin or hematocrit) is the most reliable for demonstrating increased red cell volume and for monitoring response to therapy; also, the role of bone marrow biopsy is being revisited. PV is associated with reduced survival because of cardiovascular complications and progression to post-PV myelofibrosis or leukemia. Criteria for risk-adapted treatment rely on the likelihood of thrombosis. Controlled trials have demonstrated that incidence of cardiovascular events is reduced by sustained control of hematocrit with phlebotomies (low-risk patients) and/or cytotoxic agents (high-risk patients) and antiplatelet therapy with aspirin. Hydroxyurea and interferon may be used as first-line treatments, whereas busulfan is reserved for patients that are refractory or resistant to first-line agents. However, there is no evidence that therapy improves survival, and the significance of reduction of JAK2 mutated allele burden produced by interferon is unknown. PV is also associated with a plethora of symptoms that are poorly controlled by conventional therapy. This article summarizes my approach to the management of PV in daily clinical practice.
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masked Polycythemia vera diagnosed according to who and bcsh classification
American Journal of Hematology, 2014Co-Authors: Tiziano Barbui, Maria Luigia Randi, Alessandro M Vannucchi, Elisa Rumi, Alessandra Carobbio, Guido Finazzi, Heinz Gisslinger, Jurgen Thiele, Bettina Gisslinger, Leonhard MullauerAbstract:Polycythemia vera (PV) is currently diagnosed by the World Health Organization (WHO) criteria regarding hemoglobin (HB) levels and JAK2V617F and related mutations or by the British Committee for Standards in Haematology (BCSH) guidelines predominantly based on hematocrit (HCT) values (>52% in men and >48% in women) in JAK2 mutated patients. We examined clinical features at diagnosis and outcome in 397 mutated PV patients showing a bone marrow (BM) morphology conforming with the WHO descriptions but including also cases with a HB level 65 years and white blood cell count >15 × 10(9) /L. Without these risk factors mPV patients had the same survival as overt PV suggesting that a fraction of patients with HB lower than that required for WHO diagnosis should still be considered as overt PV. This study has established the existence of mPV by two different classification systems based on either HB or HCT threshold values.
Guido Finazzi - One of the best experts on this subject based on the ideXlab platform.
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white blood cell counts and thrombosis in Polycythemia vera a subanalysis of the cyto pv study
Blood, 2015Co-Authors: Tiziano Barbui, Guido Finazzi, Arianna Masciulli, Maria Rosa Marfisi, Giovanni Tognoni, Alessandro Rambaldi, Alessandro M VannucchiAbstract:To the editor: The pathogenesis of thrombosis in patients with Polycythemia vera (PV) results from a complex interplay of patient- and disease-related variables. According to age and previous thrombosis, patients are traditionally stratified as “high risk” or “low risk.” However, recently,
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in contemporary patients with Polycythemia vera rates of thrombosis and risk factors delineate a new clinical epidemiology
Blood, 2014Co-Authors: Tiziano Barbui, Maria Luigia Randi, Elisa Rumi, Alessandra Carobbio, Guido Finazzi, Francesco Rodeghiero, Alessandro Rambaldi, Heinz Gisslinger, Bettina Gisslinger, Lisa PieriAbstract:To the editor: Controlled studies in Polycythemia vera (PV) have demonstrated the value of aggressive phlebotomy, aspirin, and cytoreductive therapy in preventing thrombotic complications.[1][1][⇓][2]-[3][3] In the European Collaboration on Low-Dose Aspirin in Polycythemia vera (ECLAP)
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masked Polycythemia vera diagnosed according to who and bcsh classification
American Journal of Hematology, 2014Co-Authors: Tiziano Barbui, Maria Luigia Randi, Alessandro M Vannucchi, Elisa Rumi, Alessandra Carobbio, Guido Finazzi, Heinz Gisslinger, Jurgen Thiele, Bettina Gisslinger, Leonhard MullauerAbstract:Polycythemia vera (PV) is currently diagnosed by the World Health Organization (WHO) criteria regarding hemoglobin (HB) levels and JAK2V617F and related mutations or by the British Committee for Standards in Haematology (BCSH) guidelines predominantly based on hematocrit (HCT) values (>52% in men and >48% in women) in JAK2 mutated patients. We examined clinical features at diagnosis and outcome in 397 mutated PV patients showing a bone marrow (BM) morphology conforming with the WHO descriptions but including also cases with a HB level 65 years and white blood cell count >15 × 10(9) /L. Without these risk factors mPV patients had the same survival as overt PV suggesting that a fraction of patients with HB lower than that required for WHO diagnosis should still be considered as overt PV. This study has established the existence of mPV by two different classification systems based on either HB or HCT threshold values.
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masked Polycythemia vera mpv results of an international study
American Journal of Hematology, 2014Co-Authors: Tiziano Barbui, Maria Luigia Randi, Alessandro M Vannucchi, Elisa Rumi, Alessandra Carobbio, Guido Finazzi, Heinz Gisslinger, Jurgen Thiele, Irene Betozzi, Lisa PieriAbstract:We examined the baseline features and clinical outcomes of 140 patients presenting with JAK2V617F positivity and a bone marrow morphology conforming with WHO criteria of Polycythemia vera (PV), but a hemoglobin level of 65 years and leukocyte count >10 × 109/L. Our data suggest that mPV is a heterogeneous myeloproliferative neoplasia and not necessarily an early/ pre-polycythemic form of classical PV that at onset in a small fraction of patients clinically may mimic essential thrombocythemia. On the other hand, the majority mPV may have a longer prodrome of undiagnosed PV or a disease biology akin to primary myelofibrosis-post PV myelofibrosis that could explain the worsening of outcome in comparison to overt/classical manifestations. Am. J. Hematol. 89:52–54, 2014. © 2013 Wiley Periodicals, Inc.
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cardiovascular events and intensity of treatment in Polycythemia vera
The New England Journal of Medicine, 2013Co-Authors: Roberto Marchioli, Guido Finazzi, Valerio De Stefano, Elena Maria Elli, Rossella R Cacciola, Giorgina Specchia, Riccardo Cavazzina, Daniela Cilloni, Alessandra Iurlo, Roberto LatagliataAbstract:A b s t r ac t Background Current treatment recommendations for patients with Polycythemia vera call for maintaining a hematocrit of less than 45%, but this therapeutic strategy has not been tested in a randomized clinical trial. Methods We randomly assigned 365 adults with JAK2-positive Polycythemia vera who were being treated with phlebotomy, hydroxyurea, or both to receive either more intensive treatment (target hematocrit, <45%) (low-hematocrit group) or less intensive treatment (target hematocrit, 45 to 50%) (high-hematocrit group). The primary composite end point was the time until death from cardiovascular causes or major thrombotic events. The secondary end points were cardiovascular events, cardiovascular hospitalizations, incidence of cancer, progression to myelofibrosis, myelodysplasia or leukemic transformation, and hemorrhage. An intention-to-treat analysis was performed. Results After a median follow-up of 31 months, the primary end point was recorded in 5 of 182 patients in the low-hematocrit group (2.7%) and 18 of 183 patients in the highhematocrit group (9.8%) (hazard ratio in the high-hematocrit group, 3.91; 95% confidence interval [CI], 1.45 to 10.53; P = 0.007). The primary end point plus superficial-vein thrombosis occurred in 4.4% of patients in the low-hematocrit group, as compared with 10.9% in the high-hematocrit group (hazard ratio, 2.69; 95% CI, 1.19 to 6.12; P = 0.02). Progression to myelofibrosis, myelodysplasia or leukemic transformation, and bleeding were observed in 6, 2, and 2 patients, respectively, in the low-hematocrit group, as compared with 2, 1, and 5 patients, respectively, in the high-hematocrit group. There was no significant between-group difference in the rate of adverse events. Conclusions In patients with Polycythemia vera, those with a hematocrit target of less than 45% had a significantly lower rate of cardiovascular death and major thrombosis than did those with a hematocrit target of 45 to 50%. (Funded by the Italian Medicines Agency and others; ClinicalTrials.gov number, NCT01645124, and EudraCT number, 2007–006694-91.)
Francesco Passamonti - One of the best experts on this subject based on the ideXlab platform.
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ruxolitinib versus standard therapy for the treatment of Polycythemia vera
The New England Journal of Medicine, 2015Co-Authors: Jeanjacques Kiladjian, Francesco Passamonti, Martin Griesshammer, Tamas Masszi, Simon Durrant, Fabrizio Pane, Pierre Zachee, Ruben A MesaAbstract:Background Ruxolitinib, a Janus kinase (JAK) 1 and 2 inhibitor, was shown to have a clinical benefit in patients with Polycythemia vera in a phase 2 study. We conducted a phase 3 open-label study to evaluate the efficacy and safety of ruxolitinib versus standard therapy in patients with Polycythemia vera who had an inadequate response to or had unacceptable side effects from hydroxyurea. Methods We randomly assigned phlebotomy-dependent patients with splenomegaly, in a 1:1 ratio, to receive ruxolitinib (110 patients) or standard therapy (112 patients). The primary end point was both hematocrit control through week 32 and at least a 35% reduction in spleen volume at week 32, as assessed by means of imaging. Results The primary end point was achieved in 21% of the patients in the ruxolitinib group versus 1% of those in the standard-therapy group (P<0.001). Hematocrit control was achieved in 60% of patients receiving ruxolitinib and 20% of those receiving standard therapy; 38% and 1% of patients in the two g...
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how i treat Polycythemia vera
Blood, 2012Co-Authors: Francesco PassamontiAbstract:Polycythemia vera (PV) is a clonal disorder characterized by unwarranted production of red blood cells. In the majority of cases, PV is driven by oncogenic mutations that constitutively activate the JAK-STAT signal transduction pathway, such as JAK2 V617F, or exon 12 mutations or LNK mutations. Diagnosis of PV is based on the WHO criteria. Diagnosis of post-PV myelofibrosis is established according to the International Working Group for Myeloproliferative Neoplasms Research and Treatment criteria. Different clinical presentations of PV are discussed. Prognostication of PV is tailored to the most frequent complication during follow-up, namely, thrombosis. Age older than 60 years and prior history of thrombosis are the 2 main risk factors for disease stratification. Correlations are emerging between leukocytosis, JAK2(V617F) mutation, BM fibrosis, and different outcomes of PV, which need to be confirmed in prospective studies. In my practice, hydroxyurea is still the "gold standard" when cytoreduction is needed, even though pegylated IFN-alfa-2a and ruxolitinib might be useful in particular settings. Results of phase 1 or 2 studies concerning these latter agents should however be confirmed by the ongoing randomized phase 3 clinical trials. In this paper, I discuss the main problems encountered in daily clinical practice with PV patients regarding diagnosis, prognostication, and therapy.
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initial bone marrow reticulin fibrosis in Polycythemia vera exerts an impact on clinical outcome
Blood, 2012Co-Authors: Tiziano Barbui, Francesco Passamonti, Filippo Marino, Irene Bertozzi, Maria Luigia Randi, J Thiele, Alessandro M Vannucchi, Elisa Rumi, Emanuela Boveri, Lisa PieriAbstract:We examined the prevalence and prognostic relevance of bone marrow reticulin fibrosis in 526 patients with World Health Organization–defined Polycythemia vera evaluated at the time of initial diagnosis. Seventy-four patients (14%) displayed mostly grade 1 reticulin fibrosis, with only 2 cases showing higher-grade fibrosis. Presenting clinical and laboratory characteristics, including JAK2V617F allele burden, between patients with and without fibrosis were similar for the most part, with the exception of a higher prevalence of palpable splenomegaly in patients with fibrosis ( P < .01). Patients with fibrosis were less prone to experience thrombosis during their clinical course (1.1 vs 2.7 per 100 patient-years; P = .03) and more prone to develop post-Polycythemia vera myelofibrosis (2.2 vs 0.8 per 100 patient-years; P = .01). There was no significant difference between the 2 groups in terms of overall or leukemia-free survival. The present study clarifies the incidence, degree, and prognostic relevance of bone marrow fibrosis obtained at time of initial diagnosis of Polycythemia vera.
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proposed criteria for the diagnosis of post Polycythemia vera and post essential thrombocythemia myelofibrosis a consensus statement from the international working group for myelofibrosis research and treatment
Leukemia, 2008Co-Authors: G Barosi, Srdan Verstovsek, Juergen Thiele, Francisco Cervantes, Peter J Campbell, Brigitte Dupriez, Ross L Levine, Francesco PassamontiAbstract:Proposed criteria for the diagnosis of post-Polycythemia vera and post-essential thrombocythemia myelofibrosis: a consensus statement from the international working group for myelofibrosis research and treatment
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life expectancy and prognostic factors for survival in patients with Polycythemia vera and essential thrombocythemia
The American Journal of Medicine, 2004Co-Authors: Francesco Passamonti, Elisa Rumi, Ester Pungolino, Lucia Malabarba, Paola Bertazzoni, Marina Valentini, Ester Orlandi, Luca Arcaini, Ercole Brusamolino, Cristiana PascuttoAbstract:Abstract Purpose To assess life expectancy and prognostic factors for survival in patients with Polycythemia vera and essential thrombocythemia. Methods The study sample consisted of 831 consecutive patients with Polycythemia vera (n = 396; 4184 person-years of follow-up) or essential thrombocythemia (n = 435; 4304 person-years of follow-up). Mortality in each group was compared with the Italian population using the standardized mortality ratio (SMR) based on life expectancy data obtained from the Italian Institute of Statistics. Results The 15-year survival was 65% in patients with Polycythemia and 73% in those with thrombocythemia. By Cox regression analysis, the independent predictors of death were a history of thrombosis for Polycythemia (hazard ratio [HR] = 2.2; P = 0.0002) and thrombocythemia (HR = 2; P = 0.01), and male sex (HR = 1.8; P = 0.03) for thrombocythemia. Mortality compared with the general population was 1.6-fold higher (P Conclusion Life expectancy of patients with Polycythemia vera (especially if younger than 50 years) was reduced compared with the general population, whereas life expectancy of patients with essential thrombocythemia was not affected significantly by the disease, reflecting the more indolent nature of the proliferation. History of thrombosis was the main predictor of death in both diseases.
Ayalew Tefferi - One of the best experts on this subject based on the ideXlab platform.
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Polycythemia vera treatment algorithm 2018
Blood Cancer Journal, 2018Co-Authors: Ayalew Tefferi, Alessandro M Vannucchi, Tiziano BarbuiAbstract:Recently reported mature survival data have confirmed the favorable prognosis in Polycythemia vera (PV), with 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. Even more discouraging has been the lack of critical appraisal from “opinion leaders”, on the added value of newly approved drugs. Keeping these issues in mind, at present, we continue to advocate conservative management in low-risk PV (phlebotomy combined with once- or twice-daily aspirin therapy) and include cytoreductive therapy in “high-risk” patients; in the latter regard, our first, second, and third line drugs of choice are hydroxyurea, pegylated interferon-α and busulfan, respectively. 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.
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tet2 mutations and their clinical correlates in Polycythemia vera essential thrombocythemia and myelofibrosis
Leukemia, 2009Co-Authors: Ayalew Tefferi, Omar Abdelwahab, Animesh Pardanani, Kenhong Lim, Terra L Lasho, Jay P Patel, Naseema Gangat, Christy Finke, Susan M SchwagerAbstract:TET2 mutations and their clinical correlates in Polycythemia vera, essential thrombocythemia and myelofibrosis
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leukocytosis as a major thrombotic risk factor in patients with Polycythemia vera commentary
Blood, 2007Co-Authors: Ayalew Tefferi, Tiziano Barbui, Guido Finazzi, Valerio De Stefano, Giovanni Tognoni, Raffaele Landolfi, Leonardo Di Gennaro, Rosamaria Marfisi, Roberto MarchioliAbstract:In Polycythemia vera, vascular risk assessment is based on age and thrombotic history, while the role of other potential predictors of this risk is still uncertain. Thus, we exploited the large database collected by the observational study of the European Collaboration on Low-Dose Aspirin in Polycythemia vera (ECLAP) to investigate the association of hematologic variables and cardiovascular risk factors with the thrombotic risk. Among 1638 polycythemic patients followed for 2.7 ±1.3 years, there were 205 thromboses. Subjects with hypertension had a mild nonsignificant increase in the risk of arterial thrombosis, while this risk was significantly increased by smoking (hazard ratio [HR], 1.90; 95% confidence interval [Cl], 1.15-3.14; P=.012). The time-dependent analysis adjusted for potential confounders showed that patients with a white blood cell count above 15 x 10 9 /L, compared with those with a white blood cell count below 10 x 10 9 /L, had a significant increase in the risk of thrombosis (HR, 1.71; 95% Cl, 1.10-2.65; P=.017), mainly deriving from an increased risk of myocardial infarction (HR, 2.84; 95% Cl, 1.25-6.46; P = .013). Thus, leukocyte count may help in defining the vascular risk of polycythemic subjects.
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anagrelide associated cardiomyopathy in Polycythemia vera and essential thrombocythemia
Haematologica, 2004Co-Authors: Donald J Jurgens, Alvaro Morenoaspitia, Ayalew TefferiAbstract:A comprehensive database inquiry at our institutions identified 11 patients with echocardiogram-documented idiopathic cardiomyopathy that post-dated a diagnosis of either Polycythemia vera or essential thrombocythemia. Anagrelide therapy was temporally associated with the particular complication in 6 patients, all of whom experienced symptomatic and/or objective improvement after drug discontinuation.
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Polycythemia vera a comprehensive review and clinical recommendations
Mayo Clinic proceedings, 2003Co-Authors: Ayalew TefferiAbstract:More than a century has elapsed since the appearance of the modern descriptions of Polycythemia vera (PV). During this time, much has been learned regarding disease pathogenesis and PV-associated molecular aberrations. New information has allowed amendments to traditional diagnostic criteria. Phlebotomy remains the cornerstone treatment of PV, whereas myelosuppressive agents may augment the benefit of using phlebotomy for thrombosis prevention in high-risk patients. Excessive aspirin use is contraindicated in PV, although the use of lower-dose aspirin has been shown to be safe and effective in alleviating microvascular symptoms including erythromelalgia and headaches. Recent studies have shown the utility of selective serotonin receptor antagonists for treating PV-associated pruritus. Nevertheless, many questions remain unanswered. What is the specific genetic mutation or altered molecular pathway that is causally related to the disease? In the absence of a specific molecular marker, how is a working diagnosis of PV made? What evidence supports current practice in the management of PV? This article summarizes both old and new information on PV; proposes a modern diagnostic algorithm to formulate a working diagnosis; and provides recommendations for patient management, relying whenever possible on an evidence-based approach.