Longitudinal follow-up of patients with thalassaemia intermedia who started transfusion therapy in adulthood: a cohort study.
Adolescent
Adult
Aged
Bilirubin
/ blood
Blood Platelets
/ metabolism
Blood Transfusion
Bone Diseases, Metabolic
/ blood
Child
Erythroblasts
/ metabolism
Female
Follow-Up Studies
Humans
Hypothyroidism
/ blood
L-Lactate Dehydrogenase
/ blood
Longitudinal Studies
Magnetic Resonance Imaging
Male
Middle Aged
Uric Acid
/ blood
beta-Thalassemia
/ blood
blood transfusions
complications
iron overload
thalassaemia
Journal
British journal of haematology
ISSN: 1365-2141
Titre abrégé: Br J Haematol
Pays: England
ID NLM: 0372544
Informations de publication
Date de publication:
10 2020
10 2020
Historique:
received:
13
01
2020
accepted:
24
04
2020
pubmed:
20
5
2020
medline:
17
3
2021
entrez:
20
5
2020
Statut:
ppublish
Résumé
We longitudinally evaluated the effects of regular blood transfusions (BTs), in the real-life context of the Myocardial Iron Overload in Thalassaemia network, in patients with thalassaemia intermedia (TI). We considered 88 patients with TI (52 females) who started regular BTs after the age of 18 years. Magnetic resonance imaging was used to quantify iron overload and biventricular function. For 56·8% of the patients there were more than two indications for the transition to regular BTs, with anaemia present in 94·0% of the cases. A significant decrease in nucleated red blood cells, platelets, lactate dehydrogenase, bilirubin, and uric acid levels was detected 6 months after starting regular BTs. After the transition to the regular BT regimen there was a significant increase only in the frequency of hypothyroidism and osteopenia, and a significant decrease in liver iron and cardiac index. The percentage of chelated patients increased significantly after starting regular BTs. The decision to regularly transfuse patients with TI may represent a way to prevent or slow down the natural progression of the disease, despite the more complex initial management.
Substances chimiques
Uric Acid
268B43MJ25
L-Lactate Dehydrogenase
EC 1.1.1.27
Bilirubin
RFM9X3LJ49
Types de publication
Journal Article
Multicenter Study
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
107-114Subventions
Organisme : Chiesi Farmaceutici S.p.A.
Organisme : ApoPharma Inc.
Informations de copyright
© 2020 British Society for Haematology and John Wiley & Sons Ltd.
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