Complications and Causes of Death in Polycythaemia Vera

Chievitz, Eva; Thiede, T

doi:10.1111/j.0954-6820.1962.tb07186.x

Cited by 255 publications

(52 citation statements)

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“…In a retrospective study, aspirin use has also been reported to be beneficial in JAK2V617F-positive low-risk ET, in preventing venous thrombosis, and also in patients with cardiovascular risk factors, in preventing arterial thrombosis [71]. There is now both controlled [40] and uncontrolled [72] evidence that supports phlebotomy for all patients with PV. In a recent randomized study, 365 adult patients with PV were treated with a target hematocrit of <45% or 45-50% [40], after a median follow-up of 31 months, the primary end point of thrombotic events or deaths from cardiovascular causes was recorded in 5 of 182 patients in the low-hematocrit group (2.7%) and 18 of 183 patients in the high-hematocrit group (9.8%) (P 5 0.007), supporting the current practice of keeping the hematocrit below 45% in patients with PV.…”

Section: Management Of Low-risk Pv or Et In The Absence Of Extreme Tmentioning

confidence: 99%

Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk‐stratification and management

2015

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Disease overview: Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms, respectively characterized by erythrocytosis and thrombocytosis. Other disease features include leukocytosis, splenomegaly, thrombosis, bleeding, microcirculatory symptoms, pruritus, and risk of leukemic or fibrotic transformation. Diagnosis: PV is defined by a JAK2 mutation, whose absence, combined with normal or increased serum erythropoietin level, makes the diagnosis unlikely. Differential diagnosis in ET includes reactive thrombocytosis, chronic myeloid leukemia, and prefibrotic myelofibrosis. Janus kinase 2 (JAK2), calreticulin (CALR), or myeloproliferative leukemia virus oncogene (MPL) mutations occur in approximately 55%, 25%, and 3% of ET patients, respectively. The same molecular markers are also present in prefibrotic myelofibrosis, which needs to be morphologically distinguished from ET. Survival and leukemic/fibrotic transformation: Median survivals are ∼14 years for PV and 20 years for ET; the corresponding values for younger patients are 24 and 33 years. Life‐expectancy in ET is inferior to the control population. JAK2/CALR mutational status does not affect survival in ET. Risk factors for survival in ET and PV include advanced age, leukocytosis, and thrombosis. Leukemic transformation rates at 20 years are estimated at <10% for PV and 5% for ET; fibrotic transformation rates are slightly higher. Thrombosis risk stratification: Current risk stratification in PV and ET is designed to estimate the likelihood of recurrent thrombosis: high‐risk is defined by the presence of age >60 years or presence of thrombosis history; low‐risk is defined by the absence of both of these two risk factors. Recent data consider JAK2V617F and cardiovascular risk factors as additional risk factors. Presence of extreme thrombocytosis might be associated with acquired von Willebrand syndrome (AvWS) and, therefore, risk of bleeding. Risk‐adapted therapy: The main goal of therapy in PV and ET is to prevent thrombohemorrhagic complications. In low risk patients, this is accomplished by the use of low‐dose aspirin and phlebotomy (hematocrit target <45%) in PV. In high risk (for thrombosis) patients, treatment with hydroxyurea is additionally recommended. Treatment with busulfan or interferon‐α is usually effective in hydroxyurea failures and the additional value of JAK inhibitor therapy in such cases is limited. Screening for AvWS is recommended before administrating aspirin, in the presence of extreme thrombocytosis. Am. J. Hematol. 90:163–173, 2015. © 2014 Wiley Periodicals, Inc.

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Section: Management Of Low-risk Pv or Et In The Absence Of Extreme Tmentioning

confidence: 99%

Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk‐stratification and management

2015

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“…Median survival in PV before the era of phlebotomy was reportedly <2 years [103] and the current figure of 14 years [35] is clearly an improvement and mostly attributed to aggressive phlebotomy that keeps the hematocrit below 45%; this was confirmed in a controlled study [84]. Therefore, phlebotomy is indicated in all patients with PV.…”

Section: Polycythemia Vera and Essential Thrombocythemiamentioning

confidence: 84%

“…Phlebotomy has been the cornerstone of treatment in PV for over a century [102]. However, early retrospective studies in PV had suggested a superior median survival with myelosuppressive therapy as opposed to either no treatment (median survival 18 months) or treatment with phlebotomy alone (median survival close to 4 years) [103]. The results from the early PVSG-led clinical trials in PV favored treatment with phlebotomy alone (median survival of 12.6 years) compared to both P-32 (median survival of 10.9 years) and chlorambucil (median survival of 9.1 years); the significant difference in survival was attributed to an increased incidence of acute myeloid leukemia (AML) in patients treated with chlorambucil or P-32 [104].…”

Section: Background Information On Treatment In Myeloproliferative Nementioning

confidence: 99%

Myeloproliferative neoplasms: A decade of discoveries and treatment advances

2015

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Myeloproliferative neoplasms (MPN) are clonal stem cell diseases, first conceptualized in 1951 by William Dameshek, and historically included chronic myeloid leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). In 1960, Nowell and Hungerford discovered an invariable association between the Philadelphia chromosome (subsequently shown to harbor the causal BCR‐ABL1 mutation) and CML; accordingly, the term MPN is primarily reserved for PV, ET, and PMF, although it includes other related clinicopathologic entities, according to the World Health Organization (WHO) classification system. In 2005, William Vainchenker and others described a Janus kinase 2 mutation (JAK2V617F) in MPN and this was followed by a series of additional descriptions of mutations that directly or indirectly activate JAK‐STAT: JAK2 exon 12, myeloproliferative leukemia virus oncogene (MPL) and calreticulin (CALR) mutations. The discovery of these, mostly mutually exclusive, “driver” mutations has contributed to revisions of the WHO diagnostic criteria and risk stratification in MPN. Mutations other than JAK2, CALR and MPL have also been described in MPN and shown to provide additional prognostic information. From the standpoint of treatment, over the last 50 years, Louis Wasserman from the Unites States and Tiziano Barbui from Italy had skillfully organized and led a number of important clinical trials, whose results form the basis for current treatment strategies in MPN. More recently, allogeneic stem cell transplant, as a potentially curative treatment modality, and JAK inhibitors, as palliative drugs, have been added to the overall therapeutic armamentarium in myelofibrosis. In the current review, I will summarize the important advances made in the last 10 years regarding the science and practice of MPN. Am. J. Hematol. 91:50–58, 2016. © 2015 Wiley Periodicals, Inc.

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“…Häufigste und potenziell bedrohliche Komplikationen sind arterielle oder venöse Thromboembolien bei bis zu 40 % der Patienten [6,7]. Bei unbehandelter PV stellen sie mit 63 % der Todesfälle die häufigste Todesursache dar [8]. Probleme der Spätphase sind der Übergang in eine sekundäre Myelofibrose (MF) mit extramedullä-rer Hämatopoese und zum Teil ausgeprägter Splenomegalie und die Transformation in eine akute Leukämie.…”

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Diagnostik und Therapie der Polycythaemia vera im Jahre 2015

Lengfelder

Grießhammer

Koschmieder

2015

Dtsch med Wochenschr

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Die klassischen myeloproliferativen Neoplasien, chronische myeloische Leukämie, Polycythaemia vera (PV), essenzielle Thrombozythämie (ET) und die primäre Myelofibrose (PMF), wurden in der Vergangenheit aufgrund der klinischen Ähnlich-keit unter dem Begriff der chronischen myeloproliferativen Erkrankungen zusammengefasst [1]. Nach der Entdeckung des Philadelphia-Chromosoms und des korrespondierenden bcr-abl-Fusionsgens [2,3]

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Complications and Causes of Death in Polycythaemia Vera

Cited by 255 publications

References 2 publications

Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk‐stratification and management

Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk‐stratification and management

Myeloproliferative neoplasms: A decade of discoveries and treatment advances

Diagnostik und Therapie der Polycythaemia vera im Jahre 2015

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