Haemoglobinopathies and other rare anemias in Spain: ten years of a nationwide registry (REHem-AR)
Por:
Marco Sánchez JM, Bardón Cancho EJ, Benéitez D, Payán-Pernía S, Collado Gimbert A, Ruiz-Llobet A, Salinas JA, Sebastián E, Argilés B, Bermúdez M, Vázquez MÁ, Ortega MJ, López Rubio M, Gondra A, Uriz JJ, Morado M, Coll MT, López Duarte M, Baro M, Cervera Á, Recasens V, García Blanes C, Del Carcavilla MP, Tallon M, González Espín A, Olteanu Olteanu FC, González P, Del Mañú Pereira MM and Cela E
Publicada:
1 ago 2024
Ahead of Print:
1 may 2024
Resumen:
REHem-AR was created in 2013. The progressive implementation of neonatal screening for haemoglobinopathies in Spanish autonomous communities where the registry had not been implemented, as well as the addition of new centres during this period, has considerably increased the sample of patients covered. In this study, we update our previous publication in this area, after a follow-up of more than 5 years. An observational, descriptive, multicentre and ambispective study of adult and paediatric patients with haemoglobinopathies and rare anaemias registered in REHem was performed. The data are from a cross-sectional analysis performed on 1 June, 2023. The study population comprised 1,756 patients, of whom 1,317 had SCD, 214 had thalassaemia and 224 were diagnosed with another condition. Slightly more than one third of SCD patients (37%) were diagnosed based on neonatal bloodspot screening, and the mean age at diagnosis was 2.5 years; 71% of thalassaemia patients were diagnosed based on the presence of anaemia. Vaso-occlusive crisis and acute chest syndrome continue to be the most frequent complications in SCD. HSCT was performed in 83 patients with SCD and in 50 patients with thalassaemia. Since the previous publication, REHem-AR has grown in size by more than 500 cases. SCD and TM are less frequent in Spain than in other European countries, although the data show that rare anaemias are frequent within rare diseases. REHem-AR constitutes an important structure for following the natural history of rare anaemias and enables us to calculate investment needs for current and future treatments.
Filiaciones:
Marco Sánchez JM:
Data Manager of the Spanish Registry of Rare Haemoglobinopathies and Rare Anaemias (REHem-AR), Gregorio Marañón Health Research Institute. Section of Pediatric Hemato-Oncology.Pediatrics Service, Hospital General Universitario Gregorio Marañón, O'Donnell, 48, Madrid, Spain
CSUR Erithropathology. ERN-EuroBloodNet. CIBERER, Universidad Complutense de Madrid, Madrid, Spain
Bardón Cancho EJ:
CSUR Erithropathology. ERN-EuroBloodNet. CIBERER, Universidad Complutense de Madrid, Madrid, Spain
Section of Pediatric Hemato-Oncology. Pediatrics Service. Hospital General, Universitario Gregorio Marañón, O'Donnell, 48, Madrid, Spain
Benéitez D:
Hematology Service. Hospital Universitario Vall d'Hebron. Barcelona. ERN-Eurobloodnet, Universitat Autònoma de Barcelona, Passeig de La Vall d'Hebron, 119-129, Barcelona, Spain
Payán-Pernía S:
Hematology Service. Hospital Universitario Virgen del Rocío, Instituto de Biomedicina de Sevilla (IBiS), Av. Manuel Siurot, S/N, 41013, Seville, Spain
Collado Gimbert A:
Section of Pediatric Hemato-Oncology. Pediatrics Service, Hospital Universitario Vall d'Hebron. Barcelona, Passeig de La Vall d'Hebron, 119-129, Barcelona, Spain
Ruiz-Llobet A:
Hematology Service. Hospital Sant Joan de DéuUniversitat de Barcelona. Institut de Recerca Hospital Sant Joan de Déu. CSUR Eritropatología. ERN-EuroBloodNet, Passeig de Sant Joan de Déu, 2Esplugues de Llobregat, Barcelona, Spain
Salinas JA:
Section of Pediatric Hemato-Oncology. Pediatrics Service, Hospital Universitari Son Espases, Carretera de Valldemossa, 79, 07120, Palma, Illes Balears, Spain
Sebastián E:
Section of Pediatric Hemato-Oncology. Hospital Infantil Universitario Niño Jesús. Foundation for Biomedical Research of the Niño Jesús University Childrens Hospital, Av. de Menéndez Pelayo, 65, 28009, Madrid, Spain
Argilés B:
Section of Pediatric Hemato-Oncology. Pediatrics Service. Hospital, Universitario y Politécnico La Fe, Avinguda de Fernando Abril Martorell, 106, Valencia, Spain
Bermúdez M:
Section of Pediatric Hemato-Oncology. Pediatrics Service. Hospital Clínico, Universitario Virgen de La Arrixaca, Ctra. Madrid-Cartagena, S/N, 30120El Palmar, Murcia, Spain
Vázquez MÁ:
Section of Pediatric Hematology. Hospital Materno-Infantil Torrecárdenas, Calle Hermandad de Donantes Sangre S/N, 04009, Almería, Spain
Ortega MJ:
Section of Pediatric Hematology. Hospital, Universitario Virgen de Las Nieves, Av. de Las Fuerzas Armadas, 2, 18014, Granada, Spain
López Rubio M:
Hematology Service. Hospital Universitario Príncipe de Asturias, Carretera de Alcalá Meco S/N, 28805, Alcalá de Henares, Madrid, Spain
Gondra A:
Section of Pediatric Hemato-Oncology. Pediatrics Service, Hospital Universitario de Basurto, Universidad del País Vasco UPV/EHU, Montevideo Etorb, 18, 48013, Bilbao, Bizkaia, Spain
Uriz JJ:
Section of Pediatric Hemato-Oncology. Pediatrics Service, Hospital Universitario Donostia, , Begiristain Doktorea Pasealekua, S/N, 20014, Donostia, Gipuzkoa, Spain
Morado M:
Hematology Service. Hospital Universitario La Paz, Paseo de La Castellana 261, 28046 , Madrid, Spain
Coll MT:
Section of Pediatric Hemato-Oncology. Pediatrics Service, Hospital General de Granollers. , Carrer de Francesc Ribas, S/N, 08402, Barcelona, Granollers, Spain
López Duarte M:
Hematology Service, Hospital Universitario Marqués de Valdecilla, Av Valdecilla S/N, 39008, Santander, Cantabria, Spain
Baro M:
Section of Pediatric Hemato-Oncology. Pediatrics Service, Hospital Doce de Octubre, Avenida Córdoba S/n2, 28041, Madrid, Spain
Cervera Á:
Pediatric Service, Hospital Universitario Móstoles, C. Dr. Luis Montes, S/N, 28935, Madrid, Móstoles, Spain
Recasens V:
Hematology Service. Hospital Miguel Servet, P.º de Isabel La Católica, 1-3, 50009, Zaragoza, Spain
García Blanes C:
Section of Pediatric Hemato-Oncology. Pediatrics Service, Hospital Clínico Valencia, Av Blasco Ibáñez, 17, 46010, Valencia, Spain
Del Carcavilla MP:
Section of Pediatric Hemato-Oncology. Pediatrics Service, Complejo Hospitalario Albacete, C. Hermano Falco, 37, 02006, Albacete, Spain
Tallon M:
Section of Pediatric Hemato-Oncology. Pediatrics Service, Hospital Álvaro Cunqueiro, Estrada de Clara Campoamor, 341, 36312, Vigo, Spain
González Espín A:
Section of Pediatric Hemato-Oncology. Pediatrics Service, Complejo Hospitalario Jaén, Av Ejército Español, 10, 23007, Jaén, Spain
Olteanu Olteanu FC:
Section of Pediatric Hemato-Oncology. Pediatrics Service. Hospital, Universitario de Guadalajara, C Donante de Sangre, S/N, 19002, Guadalajara, Spain
González P:
Hospital General Universitario Gregorio Marañón, Calle O'Donnell, 48, Madrid, Spain
Del Mañú Pereira MM:
Hospital Universitario Vall d'Hebron. BarcelonaERN-EurobloodnetUniversitat Autònoma de Barcelona, Passeig de La Vall d'Hebron, 119-129, Barcelona, Spain
Cela E:
CSUR Erithropathology. ERN-EuroBloodNet. CIBERER, Universidad Complutense de Madrid, Madrid, Spain
Section of Pediatric Hemato-Oncology. Pediatrics Service. Hospital General, Universitario Gregorio Marañón, O'Donnell, 48, Madrid, Spain
Coordinator of REHem-AR. Erythropathology Working Group of the Spanish Society of Pediatric Hematology and Oncology (SEHOP), Hospital General Universitario Gregorio Marañón, Madrid, Spain
Green Submitted, hybrid
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