Leucemia mieloide crónica en España: sus características de presentación han cambiado. Sección española del registro poblacional EUTOS / Chronic myeloid leukaemia in Spain: Its presentation characteristics have changed. Spanish section of the EUTOS population-based registry

Objetivos. Pretendemos aportar datos más fiables sobre la epidemiología de la leucemia mieloide crónica (LMC) en España que los disponibles hasta la fecha. Material y métodos. El registro poblacional EUTOS, de la European LeukemiaNet, es un registro de casos nuevos de LMC en pacientes ≥ de 18 años de 22 áreas europeas. La sección española incluyó las comunidades autónomas de Madrid, Castilla-La Mancha y Aragón, en el periodo comprendido entre el 1-2-2010 y el 31-12-2012. Resultados. Se registraron 250 casos en 35 meses. La incidencia global fue de 1,08 casos/105 habitantes-año, con predominio masculino (58% de varones) y claras diferencias entre comunidades autónomas. La incidencia estandarizada por edad fue similar (global 1,04, varones 1,31, mujeres 0,81). La mediana de edad fue de 54 años. La incidencia aumentó con la edad, siendo máxima en>65 años, aunque un 31,7% de los casos aparecieron entre los 20 y los 44 años. Un 4% se diagnosticaron en fases avanzadas (2,4% en fase acelerada, 1,6% en crisis blástica), el 56% estaban asintomáticos, el 38% tenían esplenomegalia, y el score Sokal era alto en el 11% (inferior a lo previamente reflejado en la literatura). Conclusiones. La incidencia actual de LMC en España es superior a la previamente descrita y similar a la de los estudios europeos. A diferencia de las descripciones clásicas, la LMC se presenta mayoritariamente de forma asintomática, sin esplenomegalia, con menor leucocitosis y en estadios con mejor pronóstico (AU)

Objectives. To provide more reliable data on the epidemiology of chronic myeloid leukaemia (CML) in Spain than are currently available. Material and methods. The EUTOS population-based project of European LeukemiaNet is a population registry of new CML cases in patients 18 years of age or older from 22 European areas. The Spanish section included the autonomous communities of Madrid, Castilla-La Mancha and Aragon, from 1-2-2010 to 31-12-2012. Results. A total of 250 cases were recorded in 35 months. The overall incidence was 1.08 cases/105 inhabitants-year, with a predominance of men (58%) and clear differences among the communities. The incidence standardised by age was similar (overall, 1.04; men, 1.31; women, 0.81). The median age was 54 years. The incidence increased with age, reaching a peak at>65 years, although 31.7% of cases appeared between the ages of 20 and 44 years. Four percent of cases were diagnosed in advanced stages (2.4% in accelerated phase, 1.6% in blast crisis), 56% were asymptomatic, 38% had splenomegaly, and the Sokal score was high in 11% (lower than what was previously reflected in the literature). Conclusions. The current incidence of CML in Spain is higher than previously reported and similar to that of the European studies. Unlike the classical descriptions, CML presented mostly in asymptomatic form, with no splenomegaly, less leucocytosis and in stages with better prognosis (AU)

The EUTOS population-based registry: incidence and clinical characteristics of 2904 CML patients in 20 European Countries.

This population-based registry was designed to provide robust and updated information on the characteristics and the epidemiology of chronic myeloid leukemia (CML). All cases of newly diagnosed Philadelphia positive, BCR-ABL1+ CML that occurred in a sample of 92.5 million adults living in 20 European countries, were registered over a median period of 39 months. 94.3% of the 2904 CML patients were diagnosed in chronic phase (CP). Median age was 56 years. 55.5% of patients had comorbidities, mainly cardiovascular (41.9%). High-risk patients were 24.7% by Sokal, 10.8% by EURO, and 11.8% by EUTOS risk scores. The raw incidence increased with age from 0.39/100,000/year in people 20-29 years old to 1.52 in those >70 years old, and showed a maximum of 1.39 in Italy and a minimum of 0.69 in Poland (all countries together: 0.99). The proportion of Sokal and Euro score high-risk patients seen in many countries indicates that trial patients were not a positive selection. Thus from a clinical point of view the results of most trials can be generalized to most countries. The incidences observed among European countries did not differ substantially. The estimated number of new CML cases per year in Europe is about 6370.

The EUTOS prognostic score: review and validation in 1288 patients with CML treated frontline with imatinib.

The introduction of tyrosine kinase inhibitors (TKI) in the treatment of Philadelphia chromosome-positive (Ph+) chronic myeloid leukemia (CML) has revolutionized the outcome, but the prognosis of the disease is still based on prognostic systems that were developed in the era of conventional chemotherapy and interferon (IFN)-alfa. A new prognostic score including only two variables, spleen size and basophils, was developed for the prediction of complete cytogenetic response (CCyR) and progression-free survival (PFS). The score was based on a large series of patients who were enrolled in prospective multicenter studies of first-line imatinib treatment. The prognostic value of the EUTOS (European Treatment and Outcome Study for CML) score has now been tested in an independent, multicenter, multinational series of 1288 patients who were treated first-line with imatinib outside prospective studies. It was found that also in these patients, the EUTOS prognostic score was predictive for CCyR, PFS and overall survival (OS). In addition, the prognostic value of the score was reported to be significant in seven of the eight other independent studies of almost 2000 patients that were performed in Europe, the Americas and Asia. The EUTOS risk score is a valid tool for the prediction of the therapeutic effects of TKI, particularly imatinib.