Resumen
En esta actualización se tratan en detalle el resto de las neoplasias mieloproliferativas (NMP) clásicas, denominadas NMP crónicas Filadelfia (Ph) negativas: policitemia vera, trombocitemia esencial y mielofibrosis primaria, y otras menos frecuentes como son la leucemia neutrofílica crónica (LNC) y la leucemia eosinofílica crónica (LEC). Las NMP se incluyen en la clasificación ICC (International Consensus Classification) dentro de la categoría de neoplasias mieloides y leucemias agudas de forma similar a la clasificación de neoplasias hematolinfoides de la OMS revisada en 2022.
Palabras clave
Neoplasias mieloproliferativas Filadelfia negativas
Trombocitemia esencial
Policitemia vera
Mielofibrosis primaria
Leucemia neutrofílica crónica
Leucemia eosinofílica crónica
Abstract
This update discusses in detail the remaining classic myeloproliferative neoplasms (MPNs), called Philadelphia (Ph)-negative chronic MPNs. They include polycythemia vera, essential thrombocythemia, primary myelofibrosis, and other less frequent conditions such as chronic neutrophilic leukemia (CNL) and chronic eosinophilic leukemia (CEL). MPNs are included in the ICC (International Consensus Classification) in the myeloid neoplasms and acute leukemia category, similar to the WHO classification of hematolymphoid neoplasms revised in 20221–3.
Keywords
Philadelphia-negative myeloproliferative neoplasms
Essential thrombocythemia
Polycythemia vera
Primary myelofibrosis
Chronic neutrophilic leukemia
Chronic eosinophilic leukemia
Identifíquese
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Bibliografía
[1]
M. Hultcrantz , A. Ravn Landtblom, B. Andréasson, J. Samuelsson, P.W. Dickman, S.Y. Kristinsson, et al.
Incidence of myeloproliferative neoplasms – trends by subgroup and age in a population-based study in Sweden.
J Intern Med., (2020), 287 pp. 448-454
[2]
N. Szuber, M. Mudireddy, M. Nicolosi, D. Penna, R.R. Vallapureddy, T.L. Lasho, et al.
3023 Mayo Clinic patients with myeloproliferative neoplasms: Risk-stratified comparison of survival and outcomes data among disease subgroups.
Mayo Clin Proc., (2019), 94 pp. 599-610
[3]
J.C. Ianotto , N. Curto-García, M. Lauermanova, D. Radia, J.J. Kiladjian, C.N. Harrison.
Characteristics and outcomes of patients with essential thrombocythemia or polycythemia vera diagnosed before 20 years of age: a systematic review.
Haematologica., (2019), 104 pp. 1580-1588
[4]
F. Passamonti, L. Malabarba, E. Orlandi, C. Baratè, A. Canevari, E. Brusamolino, et al.
Polycythemia vera in young patients: a study on the long-term risk of thrombosis, myelofibrosis and leukemia.
Haematologica., (2003), 88 pp. 13-18
[5]
E.J. Baxter , L.M. Scott, P.J. Campbell, C. East, N. Fourouclas, S. Swanton, et al.
Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders.
Lancet., (2005), 365 pp. 1054-1061
[6]
JAK2 exon 12 mutations in polycythemia vera and idiopathic erythrocytosis.
N Engl J Med., (2007), 356 pp. 459-468
[7]
Molecular basis of the diagnosis and treatment of polycythemia vera and essential thrombocythemia.
Blood., (2006), 107 pp. 4214-4222
[8]
Polycythemia vera: historical oversights, diagnostic details, and therapeutic views.
Leukemia., (2021), 35 pp. 3339-3351
[9]
C.N. Harrison , S. Koschmieder, L. Foltz, P. Guglielmelli, T. Flindt, M. Koehler, et al.
The impact of myeloproliferative neoplasms (MPNs) on patient quality of life and productivity: results from the international MPN Landmark survey.
Ann Hematol., (2017), 96 pp. 1653-1665
[10]
S. Cerquozzi , D. Barraco, T. Lasho, C. Finke, C.A. Hanson, R.P. Ketterling, et al.
Risk factors for arterial versus venous thrombosis in polycythemia vera: a single center experience in 587 patients.
Blood Cancer J., (2017), 7 pp. 662
[11]
M. Griesshammer, J.-J. Kiladjian, C. Besses.
Thromboembolic events in polycythemia vera.
Ann Hematol., (2019), 98 pp. 1071-1082
[12]
A prospective analysis of thrombotic events in the European collaboration study on low-dose aspirin in polycythemia (ECLAP).
Pathol Biol., (2004), 52 pp. 285-288
[13]
Characteristics and clinical significance of cytogenetic abnormalities in polycythemia vera.
Haematologica., (2017), 102 pp. 1511-1518
[14]
M.F. McMullin , C.N. Harrison, S. Ali, C. Cargo, F. Chen, J. Ewing, et al.
BSH Committee. A guideline for the diagnosis and management of polycythaemia vera. A British Society for Haematology Guideline.
Br J Haematol., (2019), 184 pp. 176-191
[15]
International Consensus Classification for myeloid neoplasms at-a-glance.
Am J Hematol., (2023), 98 pp. 6-10
[16]
D.A. Arber , A. Orazi, R.P. Hasserjian, M.J. Borowitz, K.R. Calvo, H.M. Kvasnicka, et al.
International consensus classification of myeloid neoplasms and acute leukemias: integrating morphologic, clinical, and genomic data.
Blood., (2022), 140 pp. 1200-1228
[17]
The 5th edition of the World Health Organization Classification of haematolymphoid tumours: Myeloid and histiocytic/dendritic neoplasms.
Leukemia., (2022), 36 pp. 1703-1719
[18]
Leukemic transformation in myeloproliferative neoplasms: A literature review on risk, characteristics, and outcome.
Mayo Clin Proc., (2017), 92 pp. 1118-1128
[19]
A. Tefferi , E. Rumi, G. Finazzi, H. Gisslinger, A.M. Vannucchi, F. Rodeghiero, et al.
Survival and prognosis among 1545 patients with contemporary polycythemia vera: An international study.
Leukemia., (2013), 27 pp. 1874-1881
[20]
D. Barraco , S. Cerquozzi, C.A. Hanson, R.P. Ketterling, A. Pardanani, N. Gangat, et al.
Prognostic impact of bone marrow fibrosis in polycythemia vera: validation of the IWG-MRT study and additional observations.
Blood Cancer J., (2017), 7 pp. e538
[21]
L. Ronner , N. Podoltsev, J. Gotlib, M.L. Heaney, A.T. Kuykendall, C. O’Connell, et al.
Persistent leukocytosis in polycythemia vera is associated with disease evolution but not thrombosis.
Blood., (2020), 135 pp. 1696-1703
[22]
Manual de recomendaciones en enfermedades mieloproliferativas crónicas Filadelfia negativas, 3ª ed., GEMFIN,
[23]
Mutation-enhanced international prognostic systems for essential thrombocythaemia and polycythaemia vera.
Br J Haematol., (2020), 189 pp. 291-302
[24]
Leukemogenic risk of hydroxyurea therapy in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis.
Am J Hematol., (1996), 52 pp. 42-46
[25]
A. Mathur, J. Edman, L. Liang, N.W. Scott, H.G. Watson.
Skin cancer in essential thrombocythaemia and polycythaemia vera patients treated with hydroxycarbamide.
EJHaem., (2022), 3 pp. 1305-1309
[26]
G. Barosi , G. Birgegard, G. Finazzi, M. Griesshammer, C. Harrison, H. Hasselbalch, et al.
A unified definition of clinical resistance and intolerance to hydroxycarbamide in polycythaemia vera and primary myelofibrosis: results of a European LeukemiaNet (ELN) consensus process.
Br J Haematol., (2010), 148 pp. 961-963
[27]
J.J. Kiladjian , B. Cassinat, S. Chevret, P. Turlure, N. Cambier, M. Roussel, et al.
Pegylated interferon-alfa-2a induces complete hematologic and molecular responses with low toxicity in polycythemia vera.
Blood., (2008), 112 pp. 3065-3072
[28]
N Engl J Med., (2019), 381 pp. 2135-2144
[29]
Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management.
Am J Hematol., (2024), 99 pp. 697-718
[30]
E. Rumi , F. Passamonti, M.G. Della Porta, C. Elena, L. Arcaini, L. Vanelli, et al.
Familial chronic myeloproliferative disorders: clinical phenotype and evidence of disease anticipation.
J Clin Oncol Off J Am Soc Clin Oncol., (2007), 25 pp. 5630-5635
[31]
Study of the thrombopoietin receptor in essential thrombocythemia.
Leukemia., (1997), 11 pp. 1821-1916
[32]
Diagnosis, risk stratification, and response evaluation in classical myeloproliferative neoplasms.
Blood., (2017), 129 pp. 680-692
[33]
X. Cabagnols , F. Favale, F. Pasquier, K. Messaoudi, J.P. Defour, J.C. Ianotto, et al.
Presence of atypical thrombopoietin receptor (MPL) mutations in triple-negative essential thrombocythemia patients.
Blood., (2016), 127 pp. 333-342
[34]
A. Hernández-Sánchez , Á. Villaverde-Ramiro, E. Arellano-Rodrigo, M. Garrote, I. Martín, A. Mosquera-Orgueira, Spanish MPN Group (GEMFIN), et al.
The prognostic impact of non-driver gene mutations and variant allele frequency in primary myelofibrosis.
Am J Hematol., (2024), 99 pp. 755-758
[35]
T. Barbui , G. Finazzi, A. Carobbio, J. Thiele, F. Passamonti, E. Rumi, et al.
Development and validation of an International Prognostic Score of thrombosis in World Health Organization–essential thrombocythemia (IPSET-thrombosis).
Blood., (2012), 120 pp. 5128-5133
[36]
A. Álvarez-Larrán , B. Cuevas, P. Vélez, S. Noya, G. Caballero-Navarro, F. Ferrer-Marín, et al.
Application of IPSET-thrombosis in 1366 patients prospectively followed from the spanish registry of essential thrombocythemia.
Hemasphere., (2023), 7 pp. e936
[37]
Myelofibrosis biology and contemporary management.
Br J Haematol., (2020), 191 pp. 152-170
[38]
Blood., (2023), 141 pp. 1954-1970
[39]
I. Pastor-Galán , J.C. Hernández-Boluda, J.G. Correa, A. Álvarez-Larrán, F. Ferrer-Marín, J.M. Raya, et al.
Características clínico-biológicas de los pacientes con mielofibrosis: un análisis de 1000 casos del Registro Español de Mielofibrosis.
Med Clin (Barc)., (2020), 155 pp. 152-215
[40]
Primary myelofibrosis: 2023 update on diagnosis, risk-stratification, and management.
Am J Hematol., (2023), 98 pp. 801-821
[41]
Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management.
Am J Hematol., (2021), 96 pp. 145-162
[42]
F. Cervantes , B. Dupriez, A. Pereira, F. Passamonti, J.T. Reilly, E. Morra, et al.
New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment.
Blood., (2009), 113 pp. 2895-2901
[43]
F. Passamonti , V. Alessandro, C. Domenica, A. Rambaldi, E. Morra, J.J. Kiladjian, et al.
A new international multicenter-based model to predict survival in myelofibrosis secondary to polycythemia and thrombocythemia: The Mysec Prognostic Model (MYSEC-PM).
Blood., (2014), 124 pp. 1826
[44]
P. Guglielmelli, T.L. Lasho, G. Rotunno, M. Mudireddy, C. Mannarelli, M. Nicolosi, et al.
MIPSS70: Mutation-enhanced international prognostic score system for transplantation-age patients with primary myelofibrosis.
J Clin Oncol., (2018), 36 pp. 310-318
[45]
A. Mosquera-Orgueira, E. Arellano-Rodrigo, M. Garrote, I. Martín, M. Pérez-Encinas, M.T. Gómez-Casares, Spanish MPN Group (GEMFIN), et al.
Integrating AIPSS-MF and molecular predictors: A comparative analysis of prognostic models for myelofibrosis.
Hemasphere., (2024), 8 pp. e60
[46]
F. Passamonti , C.N. Harrison, R.A. Mesa, J.-J. Kiladjian, A.M. Vannucchi, S. Verstovsek.
Anemia in myelofibrosis: Current and emerging treatment options.
Crit Rev Oncol Hematol., (2022), 180 pp. 103862
[47]
A. Genthon , M. Killian, P. Mertz, P. Cathebras, S. Gimenez De Mestral, D. Guyotat, et al.
[Myelofibrosis: A review].
La Rev Med interne., (2021), 42 pp. 101-109
[48]
Efficacy, safety, and survival with ruxolitinib in patients with myelofibrosis: results of a median 3-year follow-up of COMFORT-I.
Haematologica., (2015), 100 pp. 479-488
[49]
Fedratinib: a pharmacotherapeutic option for JAK-inhibitor naïve and exposed patients with myelofibrosis.
Expert Opin Pharmacother., (2022), 23 pp. 1677-1686
[50]
Pacritinib versus best available therapy for the treatment of myelofibrosis irrespective of baseline cytopenias (PERSIST-1): an international, randomised, phase 3 trial.
Lancet Haematol., (2017), 4 pp. e225-e236
[51]
C.N. Harrison , A.M. Vannucchi, U. Platzbecker, F. Cervantes, V. Gupta, D. Lavie, et al.
Momelotinib versus best available therapy in patients with myelofibrosis previously treated with ruxolitinib (SIMPLIFY 2): a randomised, open-label, phase 3 trial.
Lancet Haematol., (2018), 5 pp. e73-e81
[52]
Myeloablative and reduced-intensity conditioned allogeneic hematopoietic stem cell transplantation in myelofibrosis: A retrospective study by the chronic malignancies working party of the European Society for Blood and Marrow Transplantation.
Biol Blood Marrow Transplant., (2019), 25 pp. 2167-2171
[53]
H. Alchalby , T. Zabelina, T. Stübig, A. van Biezen, M. Bornhäuser, P. Di Bartolomeo, et al.
Chronic malignancies working party of the European Group for Blood and Marrow Transplantation. Allogeneic stem cell transplantation for myelofibrosis with leukemic transformation: a study from the Myeloproliferative Neoplasm Subcommittee of the CMWP of the European Group for Blood and Marrow Transplantation.
Biol Blood Marrow Transplant., (2014), 20 pp. 279-281
[54]
J. Menezes , H. Makishima, I. Gómez, F. Acquadro, G. Gómez-López, O. Graña, et al.
CSF3R T618I co-occurs with mutations of splicing and epigenetic genes and with a new PIM3 truncated fusion gene in chronic neutrophilic leukemia.
Blood Cancer J., (2013), 3 pp. e158
[55]
A. Pardanani , T.L. Lasho, R.R. Laborde, M. Elliott, C.A. Hanson, R.A. Knudson, et al.
CSF3R T618I is a highly prevalent and specific mutation in chronic neutrophilic leukemia.
Leukemia., (2013), 27 pp. 1870-1873
[56]
N.C.P. Cross , Y. Hoade, W.J. Tapper, G. Carreno-Tarragona, T. Fanelli, M. Jawhar, et al.
Recurrent activating STAT5B N642H mutation in myeloid neoplasms with eosinophilia.
Leukemia., (2019), 33 pp. 415-425
[57]
S.A. Wang , R.P. Hasserjian, W. Tam, A.G. Tsai, J.T. Geyer, T.I. George, et al.
Bone marrow morphology is a strong discriminator between chronic eosinophilic leukemia, not otherwise specified and reactive idiopathic hypereosinophilic syndrome.
Haematologica., (2017), 102 pp. 1352-1360
