Irene Pastor-Galán1, Juan Carlos Hernández-Boluda2, Juan-Gonzalo Correa3, Alberto Alvarez-Larrán4, Francisca Ferrer-Marín5, José María Raya6, Rosa Ayala7, Patricia Velez8, Manuel Pérez-Encinas9, Natalia Estrada10, Valentín García-Gutiérrez11, María Laura Fox12, Angel Payer13, Ana Kerguelen14, Beatriz Cuevas15, María Antonia Durán16, María José Ramírez17, María Teresa Gómez-Casares18, María Isabel Mata-Vázquez19, Elvira Mora20, Clara Martínez-Valverde21, Elisa Arbelo22, Anna Angona23, Elena Magro24, María Luisa Antelo25, Nieves Somolinos26, Francisco Cervantes3. 1. Servicio de Hematología, Hospital Clínico Universitario-INCLIVA, Valencia, España. 2. Servicio de Hematología, Hospital Clínico Universitario-INCLIVA, Valencia, España; Departamento de Medicina, Universidad de Valencia, Valencia, España. Electronic address: hernandez_jca@gva.es. 3. Servicio de Hematología, Hospital Clínic-IDIBAPS, Universidad de Barcelona, Barcelona, España. 4. Servicio de Hematología, Hospital Clínic-IDIBAPS, Universidad de Barcelona, Barcelona, España; Servicio de Hematología, Hospital del Mar-IMIM, Barcelona, España. 5. Servicio de Hematología y Oncología Médica, Hospital Morales Meseguer-CIBERER, IMIB-Arrixaca, UCAM, Murcia, España. 6. Servicio de Hematología, Hospital Universitario de Canarias, Tenerife, España. 7. Servicio de Hematología, Hospital 12 de Octubre, Madrid, España. 8. Servicio de Hematología, Institut Català d'Oncologia, Hospital Duran i Reynals, Hospitalet de Llobregat, Barcelona, España. 9. Servicio de Hematología, Hospital Clínico Universitario, Santiago de Compostela, España. 10. Servicio de Hematología, Institut Català d'Oncologia-Hospital Germans Trias i Pujol, Instituto de Investigación Josep Carreras, Badalona, España. 11. Servicio de Hematología, Hospital Ramón y Cajal-IRYCIS, Madrid, España. 12. Servicio de Hematología, Hospital Vall d'Hebron, Barcelona, España. 13. Servicio de Hematología, Hospital Universitario Central de Asturias, Oviedo, España. 14. Servicio de Hematología, Hospital La Paz, Madrid, España. 15. Servicio de Hematología, Hospital Universitario de Burgos, Burgos, España. 16. Servicio de Hematología, Hospital Son Espases, Mallorca, España. 17. Servicio de Hematología, Hospital de Jerez, Jerez de la Frontera, Cádiz, España. 18. Servicio de Hematología, Hospital Dr Negrín, Las Palmas de Gran Canaria, España. 19. Servicio de Hematología, Hospital Costa del Sol, Marbella, España. 20. Servicio de Hematología, Hospital La Fe, IIS La Fe, Valencia, España. 21. Servicio de Hematología, Hospital de la Santa Creu i Sant Pau, Barcelona, España. 22. Servicio de Hematología, Hospital Virgen de la Macarena, Sevilla, España. 23. Servicio de Hematología, Institut Català d'Oncologia, Hospital Josep Trueta, Girona, España. 24. Servicio de Hematología, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, España. 25. Servicio de Hematología, Complejo Hospitalario de Navarra, Pamplona, España. 26. Servicio de Hematología, Hospital Universitario de Getafe, Getafe, Madrid, España.
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 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 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. CONCLUSIONS: Myelofibrosis 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.
BACKGROUND AND OBJECTIVE MYELOFIBROSIS: is an infrequent chronic myeloproliferative neoplasm. We aimed to describe the clinico-biological characteristics, treatment, and evolutive course of myelofibrosispatients 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 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. CONCLUSIONS:Myelofibrosis 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.