4.6 Article

Polycythemia vera: 2024 update on diagnosis, risk-stratification, and management

Related references

Note: Only part of the references are listed.
Review Hematology

Aspirin in essential thrombocythemia. For whom? What formulation? What regimen?

Marco Cattaneo

Summary: Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterized by abnormal platelet production. Low-dose aspirin is recommended as the standard treatment for controlling vascular events in ET patients. However, some patients may display poor responsiveness to enteric coated aspirin. Twice daily aspirin administration has shown to overcome this problem and is considered safe with a favorable risk/benefit profile. The once daily regimen can still be used in patients with normalized platelet count to minimize potential gastrointestinal discomfort.

HAEMATOLOGICA (2023)

Editorial Material Hematology

Omalizumab alleviates pruritus in myeloproliferative neoplasms

Anna Ravn Landtblom et al.

HAEMATOLOGICA (2023)

Article Oncology

Determinants of survival and retrospective comparisons of 183 clinical trial patients with myelofibrosis treated with momelotinib, ruxolitinib, fedratinib or BMS-911543 JAK2 inhibitor

Naseema Gangat et al.

Summary: This retrospective study found that different JAK2 inhibitors had varying effects on spleen and transfusion-dependent anemia in patients with high/intermediate risk myelofibrosis. Age, genetic mutations, and treatment responses were all associated with overall survival. The study also confirmed the positive impact of treatment response on short-term survival and the long-term survival benefit of allogeneic stem cell transplants.

BLOOD CANCER JOURNAL (2023)

Article Hematology

How do valence and meaning interact? The contribution of semantic control

Naseema Gangat et al.

Summary: JAK2 unmutated or non-PV erythrocytosis includes a heterogenous spectrum of hereditary and acquired entities. Diagnosis involves excluding PV through JAK2 mutation screening and distinguishing longstanding from acquired erythrocytosis based on hematocrit and hemoglobin levels. Treatment should avoid cytoreductive therapy and indiscriminate use of phlebotomy, but therapeutic phlebotomy can be considered for symptom control. Cardiovascular risk optimization and low dose aspirin are often advised.

AMERICAN JOURNAL OF HEMATOLOGY (2023)

Editorial Material Hematology

International Consensus Classification for myeloid neoplasms at-a-glance

Attilio Orazi et al.

AMERICAN JOURNAL OF HEMATOLOGY (2023)

Review Hematology

The international consensus classification of myeloid neoplasms and acute Leukemias: myeloproliferative neoplasms

Jurgen Thiele et al.

Summary: A group of international experts met to update the World Health Organization classification system for hematopoietic tumors and introduced the new International Consensus Classification (ICC) for Myeloid Neoplasms and Acute Leukemias. The focus of this review is on the ICC-2022 category of JAK2 mutation-prevalent myeloproliferative neoplasms (MPNs) and the importance of bone marrow morphology and genetic markers in disease classification and diagnostics.

AMERICAN JOURNAL OF HEMATOLOGY (2023)

Review Hematology

Momelotinib (JAK1/JAK2/ACVR1 inhibitor): mechanism of action, clinical trial reports, and therapeutic prospects beyond myelofibrosis

Ayalew Tefferi et al.

Summary: JAK2 inhibitors are a part of the treatment for MF, mainly used to improve quality of life. The only treatment that has the potential to cure or prolong survival is allogeneic stem cell transplantation. JAK inhibitors suppress inflammatory cytokines and cell proliferation to provide therapeutic effects. The FDA has approved ruxolitinib, fedratinib, and pacritinib, with momelotinib soon to be approved.

HAEMATOLOGICA (2023)

Article Oncology

Ruxolitinib Versus Best Available Therapy for Polycythemia Vera Intolerant or Resistant to Hydroxycarbamide in a Randomized Trial

Claire N. Harrison et al.

Summary: The MAJIC-PV study shows that ruxolitinib treatment benefits PV patients resistant or intolerant to hydroxycarbamide (HC-INT/RES) with superior complete response rate, event-free survival, and molecular response; importantly, it also demonstrates for the first time, to our knowledge, that molecular response is linked to event-free survival, progression-free survival, and overall survival.

JOURNAL OF CLINICAL ONCOLOGY (2023)

Article Hematology

Predictors of anemia response to momelotinib therapy in myelofibrosis and impact on survival

Naseema Gangat et al.

Summary: This study retrospectively analyzed the data of 72 myelofibrosis patients and found that anemia response was related to survival in patients treated with momelotinib.

AMERICAN JOURNAL OF HEMATOLOGY (2023)

Article Hematology

Primary myelofibrosis: 2023 update on diagnosis, risk-stratification, and management

Ayalew Tefferi

Summary: Primary myelofibrosis is a stem cell-derived disorder characterized by clonal myeloproliferation, fibrosis, and other symptoms. Diagnosis is made through bone marrow examination and genetic testing. Treatment options mainly involve drug therapy, and new agents are under investigation.

AMERICAN JOURNAL OF HEMATOLOGY (2023)

Article Hematology

A randomized phase 3 trial of interferon-α vs hydroxyurea in polycythemia vera and essential thrombocythemia

John Mascarenhas et al.

Summary: Both hydroxyurea and interferon-alpha are effective treatments for patients with essential thrombocythemia and polycythemia vera. With longer treatment, interferon-alpha is more effective in normalizing blood counts and reducing driver mutation burden, while hydroxyurea produces more histopathologic responses.

BLOOD (2022)

Article Oncology

Neutrophil-to-lymphocyte ratio is a novel predictor of venous thrombosis in polycythemia vera

Alessandra Carobbio et al.

Summary: This study investigated the neutrophil-to-lymphocyte ratio (NLR) as a predictor of thrombosis in polycythemia vera (PV). The findings showed that an NLR value of >= 5 was independently associated with venous thrombosis risk, and the risk of venous thrombosis was almost doubled in both low- and high-risk groups when NLR >= 5.

BLOOD CANCER JOURNAL (2022)

Review Hematology

Philadelphia chromosome-negative myeloproliferative neoplasms in younger adults: A critical discussion of unmet medical needs, with a focus on pregnancy

Emanuela Sant'Antonio et al.

Summary: Myeloproliferative neoplasms (MPN) in younger patients, including women of childbearing potential, present diagnostic challenges and a unique thrombotic risk, especially during pregnancy. This article discusses the areas of uncertainty and unmet medical needs in the management of younger MPN patients, with a particular focus on pregnancy. Due to the lack of evidence-based data and the clinical heterogeneity of MPN, an individualized counseling and management strategy is recommended for young patients and expectant mothers with MPN.

BLOOD REVIEWS (2022)

Article Hematology

Ruxolitinib versus best available therapy in inadequately controlled polycythaemia vera without splenomegaly (RESPONSE-2): 5-year follow up of a randomised, phase 3b study

Francesco Passamonti et al.

Summary: The 5-year results of the RESPONSE-2 study support the use of ruxolitinib as a second-line therapy for patients with inadequately controlled polycythaemia vera without splenomegaly.

LANCET HAEMATOLOGY (2022)

Letter Hematology

SF3B1 mutations in primary and secondary myelofibrosis: Clinical, molecular and prognostic correlates

Giuseppe G. Loscocco et al.

AMERICAN JOURNAL OF HEMATOLOGY (2022)

Article Hematology

International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: integrating morphologic, clinical, and genomic data

Daniel A. Arber et al.

Summary: In 2016, the WHO, Society for Hematopathology, and European Association for Haematopathology collaborated to update the classification of myeloid neoplasms and acute leukemias, advancing the field of myeloid neoplasms and acute leukemias.

BLOOD (2022)

Review Oncology

The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic/Dendritic Neoplasms

Joseph D. Khoury et al.

Summary: This paper summarizes the new WHO classification scheme for myeloid and histiocytic/dendritic neoplasms, emphasizing the refinement of diagnostic criteria and the importance of actionable biomarkers, while maintaining global applicability.

LEUKEMIA (2022)

Article Oncology

Overall survival in the SIMPLIFY-1 and SIMPLIFY-2 phase 3 trials of momelotinib in patients with myelofibrosis

Ruben Mesa et al.

Summary: JAK inhibitors are effective for myelofibrosis, but do not address anemia. Momelotinib, targeting ACVR1/ALK2, JAK1 and JAK2, shows activity against anemia, symptoms, and splenomegaly.

LEUKEMIA (2022)

Letter Hematology

Momelotinib for myelofibrosis: 12-year survival data and retrospective comparison to ruxolitinib

Ayalew Tefferi et al.

AMERICAN JOURNAL OF HEMATOLOGY (2022)

Article Oncology

Pregnancy and childbirth outcomes in women with myeloproliferative neoplasms-a nationwide population-based study of 342 pregnancies in Sweden

Anna Ravn Landtblom et al.

Summary: Pregnancy and childbirth in women with myeloproliferative neoplasms (MPN) are associated with increased risk of preterm birth and decreased risk of maternal complications compared to previous reports.

LEUKEMIA (2022)

Review Cardiac & Cardiovascular Systems

Once- versus Twice-Daily Aspirin in Patients at High Risk of Thrombotic Events: Systematic Review and Meta-Analysis

Beatrice Mainoli et al.

Summary: This study systematically evaluated the efficacy of once- versus twice-daily aspirin in conditions with increased platelet turnover. The findings indicated that twice-daily aspirin was associated with a greater antiplatelet effect compared to once-daily aspirin, especially when using the PFA-100-ADP method.

AMERICAN JOURNAL OF CARDIOVASCULAR DRUGS (2021)

Review Oncology

Interferon alpha therapy in essential thrombocythemia and polycythemia vera-a systematic review and meta-analysis

Jan Philipp Bewersdorf et al.

Summary: Interferon-alpha treatment for essential thrombocythemia and polycythemia vera shows similar efficacy and safety between peg-IFN and non-peg-IFN, making them both viable long-term treatment options for patients with these conditions.

LEUKEMIA (2021)

Review Hematology

Myeloproliferative neoplasms and pregnancy: Overview and practice recommendations

Naseemap Gangat et al.

Summary: Pregnancy in the context of myeloproliferative neoplasms poses unique challenges, with higher risk in PV compared to ET. Interferon alpha cytoreductive therapy is recommended, along with strict hematocrit control in women with PV. Further clarification is needed through prospective clinical trials.

AMERICAN JOURNAL OF HEMATOLOGY (2021)

Article Hematology

Direct oral anticoagulants (DOAC) for prevention of recurrent arterial or venous thromboembolic events (ATE/VTE) in myeloproliferative neoplasms

Karlo Huenerbein et al.

Summary: This study compared the efficacy and safety of using VKA and DOAC in MPN patients, and found that DOAC may have a lower risk of recurrence and no significant differences in bleeding risk compared to VKA. Further studies are needed before DOAC can be routinely used in MPN patients.

ANNALS OF HEMATOLOGY (2021)

Article Hematology

Ropeginterferon alfa-2b versus phlebotomy in low-risk patients with polycythaemia vera (Low-PV study): a multicentre, randomised phase 2 trial

Tiziano Barbui et al.

Summary: The study compared the efficacy and safety of ropeginterferon alfa-2b in addition to standard phlebotomy versus phlebotomy alone in low-risk patients with polycythaemia vera. Results showed a higher response rate in the experimental group, leading to a decision to stop patient accrual due to overwhelming efficacy. Additionally, there were no significant differences in adverse events between the two groups, suggesting that supplementing phlebotomy with ropeginterferon alfa-2b is a safe and effective treatment option.

LANCET HAEMATOLOGY (2021)

Article Oncology

Interferon-alpha for treating polycythemia vera yields improved myelofibrosis-free and overall survival

Ghaith Abu-Zeinah et al.

Summary: The study suggests that treatment of PV with rIFN alpha can reduce the risk of myelofibrosis and mortality, especially in high-risk patients.

LEUKEMIA (2021)

Article Oncology

Long-term safety and efficacy of givinostat in polycythemia vera: 4-year mean follow up of three phase 1/2 studies and a compassionate use program

Alessandro Rambaldi et al.

Summary: Givinostat has shown long-term efficacy and safety in patients with PV, with a high overall response rate and low rate of Grade 3 treatment-related adverse events. This supports the long-term use of givinostat in this population.

BLOOD CANCER JOURNAL (2021)

Article Oncology

JAK2V617F variant allele frequency >50% identifies patients with polycythemia vera at high risk for venous thrombosis

Paola Guglielmelli et al.

Summary: The study analyzed the impact of JAK2V617F VAF on thrombosis in PV patients, finding that VAF > 50% is an independent strong predictor of future VT, but not associated with AT. The results suggest that AT and VT may require distinct management strategies.

BLOOD CANCER JOURNAL (2021)

Article Obstetrics & Gynecology

Management and outcomes of 27 pregnancies in women with myeloproliferative neoplasms

Joelle Lapoirie et al.

JOURNAL OF MATERNAL-FETAL & NEONATAL MEDICINE (2020)

Article Hematology

Second cancers in MPN: Survival analysis from an international study

Monia Marchetti et al.

AMERICAN JOURNAL OF HEMATOLOGY (2020)

Article Medicine, General & Internal

Incidence of myeloproliferative neoplasms - trends by subgroup and age in a population-based study in Sweden

M. Hultcrantz et al.

JOURNAL OF INTERNAL MEDICINE (2020)

Letter Hematology

Mayo CALR mutation type classification guide using alpha helix propensity

Terra L. Lasho et al.

AMERICAN JOURNAL OF HEMATOLOGY (2018)

Letter Hematology

Pregnancy outcome and management of 25 pregnancies in women with polycythemia vera

Irene Bertozzi et al.

AMERICAN JOURNAL OF HEMATOLOGY (2018)

Article Medicine, General & Internal

Risk for Arterial and Venous Thrombosis in Patients With Myeloproliferative Neoplasms A Population-Based Cohort Study

Malin Hultcrantz et al.

ANNALS OF INTERNAL MEDICINE (2018)

Letter Hematology

Cytogenetic findings in WHO-defined polycythaemia vera and their prognostic relevance

Daniela Barraco et al.

BRITISH JOURNAL OF HAEMATOLOGY (2018)

Letter Hematology

The effect of arterial hypertension on thrombosis in low-risk polycythemia vera

Tiziano Barbui et al.

AMERICAN JOURNAL OF HEMATOLOGY (2017)

Article Hematology

Gender and survival in essential thrombocythemia: A two-center study of 1,494 patients

Ayalew Tefferi et al.

AMERICAN JOURNAL OF HEMATOLOGY (2017)

Article Hematology

Calreticulin variant stratified driver mutational status and prognosis in essential thrombocythemia

Yoseph C. Elala et al.

AMERICAN JOURNAL OF HEMATOLOGY (2016)

Article Hematology

Pruritus in primary myelofibrosis: management options in the era of JAK inhibitors

Brianna E. Vaa et al.

ANNALS OF HEMATOLOGY (2016)

Article Hematology

Survival after splanchnic vein thrombosis: A 20-year nationwide cohort study

Kirstine Kobberoe Sogaard et al.

THROMBOSIS RESEARCH (2016)

Article Hematology

Targeted deep sequencing in polycythemia vera and essential thrombocythemia

Ayalew Tefferi et al.

BLOOD ADVANCES (2016)

Article Medicine, General & Internal

Ruxolitinib versus Standard Therapy for the Treatment of Polycythemia Vera

Alessandro M. Vannucchi et al.

NEW ENGLAND JOURNAL OF MEDICINE (2015)

Article Medicine, General & Internal

Long-term Clinical Outcomes of Splanchnic Vein Thrombosis Results of an International Registry

Walter Ageno et al.

JAMA INTERNAL MEDICINE (2015)

Review Oncology

Myeloproliferative Neoplasms A Contemporary Review

Ayalew Tefferi et al.

JAMA ONCOLOGY (2015)

Article Hematology

Masked polycythemia vera diagnosed according to WHO and BCSH classification

Tiziano Barbui et al.

AMERICAN JOURNAL OF HEMATOLOGY (2014)

Article Medicine, General & Internal

Cardiovascular Events and Intensity of Treatment in Polycythemia Vera

Roberto Marchioli et al.

NEW ENGLAND JOURNAL OF MEDICINE (2013)

Article Cell Biology

Mouse models of myeloproliferative neoplasms: JAK of all grades

Juan Li et al.

DISEASE MODELS & MECHANISMS (2011)

Letter Hematology

Polycythemia vera-associated acquired von Willebrand syndrome despite near-normal platelet count

Ayaew Tefferi et al.

AMERICAN JOURNAL OF HEMATOLOGY (2010)

Review Medicine, General & Internal

Polycythemia vera-associated pruritus and its management

Kamal S. Saini et al.

EUROPEAN JOURNAL OF CLINICAL INVESTIGATION (2010)

Article Gastroenterology & Hepatology

Acute Portal Vein Thrombosis Unrelated to Cirrhosis: A Prospective Multicenter Follow-up Study

Aurelie Plessier et al.

HEPATOLOGY (2010)

Letter Hematology

Two rare MPL gene mutations in patients with essential thrombocythemia

Haruhiko Ohashi et al.

INTERNATIONAL JOURNAL OF HEMATOLOGY (2009)

Article Medicine, General & Internal

JAK2 exon 12 mutations in polycythemia vera and idiopathic erythrocytosis

Linda M. Scott et al.

NEW ENGLAND JOURNAL OF MEDICINE (2007)

Article Medicine, General & Internal

Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia

CN Harrison et al.

NEW ENGLAND JOURNAL OF MEDICINE (2005)

Article Medicine, General & Internal

A gain-of-function mutation of JAK2 in myeloproliferative disorders

R Kralovics et al.

NEW ENGLAND JOURNAL OF MEDICINE (2005)

Article Oncology

Vascular and neoplastic risk in a large cohort of patients with polycythemia vera

R Marchioli et al.

JOURNAL OF CLINICAL ONCOLOGY (2005)

Article Medicine, General & Internal

Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders

EJ Baxter et al.

LANCET (2005)

Review Hematology

Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia

MA Elliott et al.

BRITISH JOURNAL OF HAEMATOLOGY (2005)

Article Medicine, General & Internal

Efficacy and safety of low-dose aspirin in polycythemia vera

R Landolfi et al.

NEW ENGLAND JOURNAL OF MEDICINE (2004)

Article Dermatology

Narrowband (TL-01) ultraviolet B phototherapy for pruritus in polycythaemia vera

A Baldo et al.

BRITISH JOURNAL OF DERMATOLOGY (2002)

Article Hematology

Pruritus in polycythaemia vera: prevalence, laboratory correlates and management

F Diehn et al.

BRITISH JOURNAL OF HAEMATOLOGY (2001)