Low-risk polycythemia vera treated with phlebotomies: clinical characteristics, hematologic control and complications in 453 patients from the Spanish Registry of Polycythemia Vera
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Published:2022-08-30
Issue:10
Volume:101
Page:2231-2239
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ISSN:0939-5555
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Container-title:Annals of Hematology
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language:en
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Short-container-title:Ann Hematol
Author:
Triguero AnaORCID, Pedraza Alexandra, Pérez-Encinas Manuel, Mata-Vázquez María Isabel, Vélez Patricia, Fox Laura, Gómez-Calafat Montse, García-Delgado Regina, Gasior Mercedes, Ferrer-Marín Francisca, García-Gutiérrez Valentín, Angona Anna, Gómez-Casares María Teresa, Cuevas Beatriz, Martínez Clara, Pérez Raúl, Raya José María, Guerrero Lucía, Murillo Ilda, Bellosillo Beatriz, Hernández-Boluda Juan Carlos, Sanz Cristina, Álvarez-Larrán Alberto,
Abstract
AbstractHematological control, incidence of complications, and need for cytoreduction were studied in 453 patients with low-risk polycythemia vera (PV) treated with phlebotomies alone. Median hematocrit value decreased from 54% at diagnosis to 45% at 12 months, and adequate hematocrit control over time (< 45%) was observed in 36%, 44%, and 32% of the patients at 6, 12, and 24 months, respectively. More than 5 phlebotomies per year in the maintenance phase were required in 19% of patients. Worsening thrombocytosis, age > 60 years, and microvascular symptoms constituted the main indications for starting cytoreduction. Median duration without initiating cytoreduction was significantly longer in patients younger than 50 years (< 0.0001). The incidence rate of thrombosis under phlebotomies alone was 0.8% per year and the estimated probability of thrombosis at 10 years was 8.5%. The probability of arterial thrombosis was significantly higher in patients with arterial hypertension whereas there was a trend to higher risk of venous thrombosis in cases with high JAK2V617F allele burden. Rates of major bleeding and second primary neoplasm were low. With a median follow-up of 9 years, survival probability at 10 years was 97%, whereas the probability of myelofibrosis at 10 and 20 years was 7% and 20%, respectively. Progression to acute myeloid leukemia was documented in 3 cases (1%). Current management of low-risk PV patients is associated with low rate of thrombosis and long survival. New treatment strategies are needed for improving hematological control and, in the long term, reducing progression to myelofibrosis.
Funder
Instituto de Salud Carlos III Novartis Pharma
Publisher
Springer Science and Business Media LLC
Subject
Hematology,General Medicine
Reference20 articles.
1. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H TJ (2017) WHO classification of tumours of haematopoietic and lymphoid tissues. Revised Fourth Edition. 4th ed. IARC, editor. 2. Barbui T, Tefferi A, Vannucchi AM, Passamonti F, Silver RT, Hoffman R, Verstovsek S, Mesa R, Kiladjian JJ, Hehlmann R, Reiter A, Cervantes F, Harrison C, Mc Mullin MF, Hasselbalch HC, Koschmieder S, Marchetti M, Bacigalupo A, Finazzi G, Kroeger N, Griesshammer M, Birgegard G, Barosi G (2018) Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet. Leukemia 32(5):1057–1069. https://doi.org/10.1038/s41375-018-0077-1 3. Barbui T, Carobbio A, Rumi E, Finazzi G, Gisslinger H, Rodeghiero F, Randi ML, Rambaldi A, Gisslinger A, Gisslinger B, Pieri L, Bertozzi I, Casetti I, Pardanani A, Passamonti F, Vanucchi AM, Tefferi A (2014) In contemporary patients with polycythemia vera, rates of thrombosis and risk factors delineate a new clinical epidemiology. Blood 124:3021–3023. https://doi.org/10.1182/blood-2014-07-591610 4. Landolfi R, Di Gennaro L, Barbui T, De Stefano V, Finazzi G, Marfisi R, Tognoni G, Marchioli R (2007) Leukocytosis as a major thrombotic risk factor in patients with polycythemia vera. Blood 109(6):2446–2452 5. Alvarez-Larrán A, Bellosillo B, Pereira A, Kerguelen A, Hernández-Boluda JC, Martínez-Avilés L, Fernández-Rodríguez C, Gómez M, Lombardía L, Angona A, Ancochea A, Senín A, Longarón R, Navarro B, Collado M, Besses C (2014) JAK2V617F monitoring in polycythemia vera and essential thrombocythemia: clinical usefulness for predicting myelofibrotic transformation and thrombotic events. Am J Hematol 89(5):517–523. https://doi.org/10.1182/blood-2006-08-042515
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