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

Irene Pastor‐Galán, Juan Carlos Hernández‐Boluda, Juan Gonzalo Correa, Alberto Álvarez‐Larrán, Francisca Ferrer‐Marín, José María Raya, Rosa Ayala, Patricia Vélez, Manuel Pérez‐Encinas, Natàlia Estrada, Valentín García‐Gutiérrez, María Laura Fox, Ángel Ramírez Páyer, Ana Kerguelen, Beatriz Cuevas, María Antonia Durán, Marı́a José Ramı́rez, María Teresa Gómez‐Casares, María Isabel Mata‐Vázquez, Elvira Mora, Clara Martínez‐Valverde, Elisa Arbelo, Anna Angona, Elena Magro, María Luisa Antelo, Nieves Somolinos, Francisco Cervantes
In: Medicina Clínica (English Edition) · 2020 · vol. 155(4) , pp. 152–158 · doi:10.1016/j.medcle.2019.11.010 · PMID:31980217 · W3001840506
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This study analyzed the clinico-biological characteristics of 1,000 myelofibrosis patients from the Spanish Registry of Myelofibrosis.

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Abstract

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 methodsA total of 1,000 patients from the Spanish Registry of Myelofibrosis diagnosed with primary (n=641) or secondary (n=359) myelofibrosis were analysed.ResultsMedian 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 incidence of leukaemia at 10 years was 15%. The most frequent therapies for the anaemia were the erythropoiesis stimulating agents and danazol. From 2010, a progressive increase in the use of ruxolitinib was noticed. A total of 7.5% of patients were transplanted. During the observation period, 42% of patients died mainly due to the clinical deterioration caused by myelofibrosis or leukaemic transformation. The median survival of the series was 5.7 years. Four different risk categories were identified by the IPSS: median survival was not reached in the low risk group and was 8.8 years, 5.3 years, and 2.8 years in the intermediate-1, intermediate-2, and high-risk groups, respectively.ConclusionsMyelofibrosis is a disabling condition mainly affecting elderly people. Its treatment is mostly driven by symptom control. Despite its clinical heterogeneity, several prognostic models are useful to select candidates for transplantation.

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