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  3. Haemoglobinopathies and other rare anemias in Spain: ten years of a nationwide registry (REHem-AR).

Haemoglobinopathies and other rare anemias in Spain: ten years of a nationwide registry (REHem-AR).

Fecha de publicación: Fecha Ahead of Print:

Autores de IDIVAL

  • Mónica López Duarte

    Autor

  • Paula González García

    Autor

Autores ajenos al IDIVAL

  • 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
  • Baro M
  • Cervera Á
  • Recasens V
  • García Blanes C
  • Del Carcavilla MP
  • Tallon M
  • González Espín A
  • Olteanu Olteanu FC
  • Del Mañú Pereira MM
  • Cela E

Unidades

Abstract

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.

© 2024. The Author(s).

Datos de la publicación

ISSN/ISSNe:
0939-5555, 1432-0584

ANNALS OF HEMATOLOGY  SPRINGER

Tipo:
Article
Páginas:
2743-2755
PubMed:
38763941

Citas Recibidas en Web of Science: 2

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Keywords

  • Hemoglobinopathies; Nationwide registry; Rare anaemias

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