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Neoplasias mieloproliferativas negativas filafelfia
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Palabras clave

Neoplasias mielopoliferantes Filadelfia negativas
Hidroxiurea
Ruxolitinib

Cómo citar

Neoplasias mieloproliferativas negativas filafelfia. (2026). Revista Eletrônica Acervo Saúde, 26(10), e23873. https://doi.org/10.25248/reas.e23873.2026

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Resumen

Objetivo: Comprender en general el diagnóstico y la patogenia de las neoplasias mieloproliferativas negativas para el cromosoma Filadelfia. Revisión de la literatura: La trombosis es la amenaza más inmediata para la salud de los pacientes. Esto significa que los pacientes con neoplasias mieloproliferativas tienen un mayor riesgo de desarrollar trombosis en comparación con la población general. Los enfoques terapéuticos actuales tienen como objetivo reducir el riesgo de trombosis con agentes antiplaquetarios, flebotomía terapéutica y terapias citorreductoras como la hidroxiurea. Debido al riesgo inherente de trombosis en PV y TE, independientemente de la estratificación de riesgo actual, se ha optado por iniciar la terapia citorreductora. El descubrimiento de la mutación activadora de JAK2 (V617F), presente en más del 70 % de los pacientes con MPN, permitió el desarrollo de nuevos inhibidores bioquímicamente selectivos de JAK2. Uno de los inhibidores de JAK1/2 es ruxolitinib, que es un agente no mielotóxico que no mata las células madre hematopoyéticas. Consideraciones finales: A pesar de los avances, es necesario buscar nuevos agentes terapéuticos que ofrezcan buena acción y menores efectos secundarios.

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Referencias

ARBER DA, et al. The 2016 revision of the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391–405.

BARBUI, T. et al. Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet. Leukemia, v. 32, n. 5, p. 1057–1069, 27 fev. 2018.

BARBUI T, et al. Em pacientes contemporâneos com policitemia vera, taxas de trombose e fatores de risco delineiam uma nova epidemiologia clínica. Sangue J Am Soc Hematol. 2014;124(19):3021–3.

BARBUI T, et al. Development and validation of an International Prognostic Score of thrombosis in World Health Organization-essential thrombocythemia (IPSET-thrombosis). Blood. 2012 Dec 20;120(26):5128-33

BARBUI T, et al. Neoplasias mieloproliferativas clássicas negativas da Filadélfia: conceitos críticos e recomendações de manejo da European LeukemiaNet., J Clin Oncol,2011, vol.29 6 (pág. 761-70).

BEER P, et al. Como eu trato a trombocitemia essencial . Blood . 2011; 117 ( 5 ): 1472-82.

BITTENCOURT RI, et al. Philadelphia-negative chronic myeloproliferative neoplasms. Rev Bras Hematol Hemoter. 2012;34(2):140-9.

BITTENCOURT RI, et al. Trombocitose essencial: o que é essencial saber. Rev Bras Hematol. Hemoter. Porto Alegre. v. 32, n. 2, p. 162-70, 2010.

CASU C, et al. Minihepcidin peptides as disease modifiers in mice affected by β-thalassemia and polycythemia vera. Blood, v. 128, n. 2, p. 265–276, 14 jul. 2016.

CHAUFFAILLE MLLF. Myeloproliferative neoplasm: a review of diagnostic criteria and clinical aspects. Rev. bras. hematol. hemoter ; 32(4): 308-316, 2010.

DOAA HASSANIEN, et al. Advances in polycythemia vera treatment with targeted therapies and clinical trials. Discover Oncology, v. 16, n. 1, 27 out. 2025.

FINAZZI G, et al A phase II study of Givinostat in combination with hydroxycarbamide in patients with polycythaemia vera unresponsive to hydroxycarbamide monotherapy. Br J Haematol. 2013 Jun;161(5):688-94.

GENTHON A, et al. Les myélofibroses [Myelofibrosis: A review]. Rev Med Interne. 2021 Feb;42(2):101-109.

GISSLINGER H, et al. Ropeginterferon alfa-2b versus terapia padrão para policitemia vera (PROUD-PV e CONTINUATION-PV): um estudo de fase 3 randomizado de não inferioridade e seu estudo de extensão. Lanceta Hematol. 2020;7(3):e196–208.

GISSLINGER H, et al. Ropeginterferon alfa-2b, a novel IFNα-2b, induces high response rates with low toxicity in patients with polycythemia vera. Blood J Am Soc Hematol. 2015;126(15):1762–9.

GREENFIELD G, et al. Patogênese molecular das neoplasias mieloproliferativas. J Hematol Oncol. 30 de junho de 2021;14(1):103.

GRIESSHAMMER M, et al. Ruxolitinib for the treatment of inadequately controlled polycythemia vera without splenomegaly: 80-week follow-up from the RESPONSE-2 trial.. Ann Hematol. 2018;97:1591–600.

GUPTA V, et al Genetic factors rather than blast reduction determine outcomes of allogeneic HCT in BCR-ABL-negative MPN in blast phase. Blood Adv. 2020;4(21):5562–73.

HARRISON CN, et al. Long-term findings from COMFORT-II, a phase 3 study of ruxolitinib vs best available therapy for myelofibrosis. Leukemia. 2016 Aug;30(8):1701-7.

HARRISON CN, et al. Janus kinase-2 inhibitor fedratinib in patients with myelofibrosis previously treated with ruxolitinib (JAKARTA-2): a single-arm, open-label, non-randomised, phase 2, multicentre study. Lancet Haematol. 2017 Jul;4(7):e317-e324.

HULTCRANTZ M, et al. Risco de trombose arterial e venosa em 11.155 pacientes com neoplasias mieloproliferativas e 44.620 controles pareados; um estudo de base populacional. Washington, DC: Sociedade Americana de Hematologia; 2014.

KENNEDY JA, et al. Treatment outcomes following leukemic transformation in Philadelphia-negative myeloproliferative neoplasms. Blood. 2013 Apr;121(14):2725-2733.

KING KY, et al. Comparative long-term effects of interferon α and hydroxyurea on human hematopoietic progenitor cells. Experimental Hematology, v. 43, n. 10, p. 912-918.e2, 1 out. 2015.

LIU Y, et al. The genomic landscape of pediatric and young adult T-lineage acute lymphoblastic leukemia. Nat Genet. 2017 Aug;49(8):1211-1218.

LUSSANA F, et al Association of V617F Jak2 mutation with the risk of thrombosis among patients with essential thrombocythaemia or idiopathic myelofibrosis: a systematic review. Thromb Res. 2009 Sep;124(4):409-17.

MARCHIOLI R, et al. The CYTO-PV: A Large-Scale Trial Testing the Intensity of CYTO reductive Therapy to Prevent Cardiovascular Events in Patients with Polycythemia Vera. Thrombosis. 2011;2011:794240.

MARCHIOLI R, et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013 Jan 3;368(1):22-33.

MORA B, et al. Patients with post-essential thrombocythemia and post-polycythemia vera differ from patients with primary myelofibrosis. Leuk Res. 2017 Aug;59:110-116.

MUSSO, A. Tratamiento de las Neoplasias Mieloproliferativas BCR-ABL1 Negativas (NMP). Experiencia Personal con Interferón y Comentarios sobre Nuevas Drogas. Revista Hematología, [S. l.], v. 25, n. 2, p. 75–84, 2021. Disponível em: https://mail.revistahematologia.com.ar/index.php/Revista/article/view/369.

NANGALIA J, et al. Mutações somáticas de CALR em neoplasias mieloproliferativas com JAK2 não mutado. N Engl J Med. 2013;369(25):2391–405.

NURGAT Z, LAWRENCE M. Management of Myeloproliferative Neoplasms (MPNs). J Oncol Pharm Pract. 2022 Sep;28(6):1400-1410.

PASSAMONTI F, et al. Ruxolitinib for the treatment of inadequately controlled polycythaemia vera without splenomegaly (RESPONSE-2): a randomised, open-label, phase 3b study. Lancet Oncol. 2017 Jan;18(1):88-99.

PATRONO C, et al. Platelet activation and inhibition in polycythemia vera and essential thrombocythemia. Blood. 2013 Mar 7;121(10):1701-11.

PLOU E, et al. JAK2 stimulates homologous recombination and genetic instability: potential implication in the heterogeneity of myeloproliferative disorders. Blood. 2008 Aug 15;112(4):1402-12.

RAMBALDI A, et al. Safety and efficacy of the maximum tolerated dose of givinostat in polycythemia vera: a two-part Phase Ib/II study. Leukemia. 2020 Aug;34(8):2234-2237.

RAMPAL R, et al. Integrated genomic analysis illustrates the central role of JAK-STAT pathway activation in myeloproliferative neoplasm pathogenesis. Blood. 2014 May 29;123(22):e123-33.

SCOTT LM, et al. JAK2 exon 12 mutations in polycythemia vera and idiopathic erythrocytosis. N Engl J Med. 2007 Feb 1;356(5):459-68.

SPIVAK JL, et al. Dois fenótipos clínicos na policitemia vera. N Engl J Med. 2014; 371(9):808-817.

THAPA B, et al. Myeloproliferative Neoplasms. [Updated 2023 Aug 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2026 Jan-.

TIACCI E, et al. Pervasive mutations of JAK-STAT pathway genes in classical Hodgkin lymphoma. Blood. 2018 May 31;131(22):2454-2465.

VANNUCCHI AM, et al. Ruxolitinibe versus terapia padrão para o tratamento da policitemia vera. N Engl J Med. 2015;372(5):426–35.

VENUGOPAL S, MASCARENHAS J. New therapies in myeloproliferative neoplasms. Hematol Oncol 13 , 162 (2020).

VERSTOVSEK S, et al. Um estudo duplo-cego, controlado por placebo de ruxolitinib para mielofibrose. N Engl J Med. 2012;366(9):799–807.

WINGELHOFER B, et al. Implications of STAT3 and STAT5 signaling on gene regulation and chromatin remodeling in hematopoietic cancer. Leukemia. 2018 Aug;32(8):1713-1726.

YOON SY, WON, J-H The clinical role of interferon alpha in Philadelphia-negative myeloproliferative neoplasms. Blood Research, v. 56, n. S1, p. S44–S50, 30 abr. 2021.