Early initiation of disease-modifying therapy can impede or prevent diffuse myocardial fibrosis in sickle cell anemia

Cardiovascular disease is a major cause of mortality in patients with sickle cell disease (SCD). Niss et al previously reported that cardiac magnetic resonance in 25 patients showed universal myocardial fibrosis, which they correlated with increased extracellular volume fraction (ECV). In the curren...

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Bibliographic Details
Published in:Blood 2022-09, Vol.140 (11), p.1322-1324
Main Authors: Niss, Omar, Detterich, Jon, Wood, John C, Coates, Thomas D, Malik, Punam, Taylor, Michael D, Quinn, Charles T
Format: Article
Language:English
Subjects:
Anemia, Sickle Cell - complications
Anemia, Sickle Cell - drug therapy
Anemia, Sickle Cell - pathology
Cardiomyopathies - pathology
Fibrosis
Humans
Letter to Blood
Myocardium - pathology
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Summary:Cardiovascular disease is a major cause of mortality in patients with sickle cell disease (SCD). Niss et al previously reported that cardiac magnetic resonance in 25 patients showed universal myocardial fibrosis, which they correlated with increased extracellular volume fraction (ECV). In the current study, they compared patients with SCD who were treated with hydroxyurea or transfusion at age
ISSN:0006-4971
1528-0020
DOI:10.1182/blood.2021015303