0000000000303448

AUTHOR

María Teresa Gómez-casares

Características clínico-biológicas de los pacientes con mielofibrosis: un análisis de 1.000 casos del Registro Español de Mielofibrosis

Resumen Antecedentes y objetivo La mielofibrosis es una neoplasia mieloproliferativa cronica infrecuente. Nuestro objetivo fue describir las caracteristicas clinico-biologicas, el tratamiento y el curso evolutivo de los pacientes con mielofibrosis en Espana. Material y metodos Se analizaron 1.000 pacientes del Registro Espanol de Mielofibrosis diagnosticados de mielofibrosis primaria (n = 641) o secundaria (n = 359). Resultados La mediana de edad era de 68 anos. La frecuencia de sintomatologia constitucional, anemia moderada o severa (Hb  Conclusiones la mielofibrosis es una enfermedad invalidante que afecta sobre todo a personas de edad avanzada y cuyo tratamiento es fundamentalmente sinto…

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rs2431697, a Polymorphism of Mir-146a, Is a Precozing Marker of Progression to Secondary Myelofibrosis: New Epigenetic Regulation of Jak/Stat3 Signaling

Abstract Introduction: Transformation to secondary myelofibrosis (MF) occurs as part of the natural history of polycythemia vera (PV) and essential thrombocythemia (ET), the two more indolent Ph-negative myeloproliferative neoplasms (MPN). Once transformed, survival is remarkably shorted. Chronic inflammation plays a critical role in the progression of MPN, driving clonal expansion toward end stage disease. Importantly, MPN are characterized by the production of inflammatory cytokines, by both malignant and non-malignant clone. Inflammation and cancer share a common pathway, i.e. NF-κB. Interestingly, miR-146a regulates TLR/NF-κB pathway through the inhibition of its targets, IRAK1 and TRAF…

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Impact of Sars-CoV2 Infection on 491 Hematological Patients: The Ecovidehe Multicenter Study

INTRODUCTION Coronavirus disease 2019 (COVID-19) caused by SARS-CoV2 virus is thought to be more severe in patients with prior hematological diseases. There is evidence suggesting that hematological patients are particularly vulnerable and have a higher risk of developing severe events, with higher mortality rate than general population. However, the available data are limited, and prognostic factors at admission still remain unclear. With this background, our aims were to analyze the impact of hematological diseases and their therapy on the COVID-19 severity and to identify clinical and biological risk factors to predict the outcome in these patients. METHODS We carried out a multicenter …

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Prognostic value of FLT3 mutations in patients with acute promyelocytic leukemia treated with all-trans retinoic acid and anthracycline monochemotherapy

Background Fms-like tyrosine kinase-3 (FLT3) gene mutations are frequent in acute promyelocytic leukemia but their prognostic value is not well established. Design and Methods We evaluated FLT3-internal tandem duplication and FLT3-D835 mutations in patients treated with all-trans retinoic acid and anthracycline-based chemotherapy enrolled in two subsequent trials of the Programa de Estudio y Tratamiento de las Hemopatias Malignas (PETHEMA) and Hemato-Oncologie voor Volwassenen Nederland (HOVON) groups between 1996 and 2005. Results FLT3-internal tandem duplication and FLT3-D835 mutation status was available for 306 (41%) and 213 (29%) patients, respectively. Sixty-eight (22%) and 20 (9%) pa…

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Clinico-biological characteristics of patients with myelofibrosis: an analysis of 1,000 cases from the Spanish Registry of Myelofibrosis

BACKGROUND AND OBJECTIVE MYELOFIBROSIS: is an infrequent chronic myeloproliferative neoplasm. We aimed to describe the clinico-biological characteristics, treatment, and evolutive course of myelofibrosis patients in Spain.; MATERIAL AND METHODS: A total of 1,000 patients from the Spanish Registry of Myelofibrosis diagnosed with primary (n=641) or secondary (n=359) myelofibrosis were analysed.; RESULTS: Median age was 68 years. The frequency of constitutional symptoms, moderate to severe anaemia (Hb<10g/dL), and symptomatic splenomegaly was 35%, 36%, and 17%, respectively. The rate of thrombosis and haemorrhage was 1.96 and 1.6 events per 100 patient-years, respectively. The cumulative incid…

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Excess Mortality in Polycythemia Vera and Essential Thrombocythemia

Abstract Background and objective. An important proportion of patients with polycythemia vera (PV) and essential thrombocythemia (ET) are diagnosed in the seventh and eighth decades of life. Because of the chronic course of PV and ET and the advanced age of patients, many will actually die from age-related ailments instead of the myeloproliferative neoplasm, so the disease's impact on life-expectancy remains largely unknown. This registry‐based study was aimed at investigating the excess mortality attributable to PV and ET in a large series of patients diagnosed and managed according to modern criteria. Methods. We queried the databases of the Spanish Group for Chronic Myeloproliferative Ne…

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