Diffuse myocardial fibrosis occurs in young patients with sickle cell anemia despite early disease-modifying therapy

Abstract

Letter to Blood| March 16, 2023 Diffuse myocardial fibrosis occurs in young patients with sickle cell anemia despite early disease-modifying therapy Clinical Trials & Observations Cara E. Morin, Cara E. Morin ∗ 1Department of Radiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH2Department of Diagnostic Imaging, St. Jude Children’s Research Hospital, Memphis, TN https://orcid.org/0000-0003-1953-4486 Search for other works by this author on: This Site PubMed Google Scholar Akshay Sharma, Akshay Sharma ∗ 3Department of Bone Marrow Transplantation and Cellular Therapy, St. Jude Children’s Research Hospital, Memphis, TN https://orcid.org/0000-0003-3281-2081 Search for other works by this author on: This Site PubMed Google Scholar Subodh Selukar, Subodh Selukar 4Department of Biostatistics, St. Jude Children’s Research Hospital, Memphis, TN https://orcid.org/0000-0002-3802-488X Search for other works by this author on: This Site PubMed Google Scholar Gary Beasley, Gary Beasley 5Division of Pediatric Cardiology, University of Tennessee Health Science Center, Le Bonheur Children’s Hospital, Memphis, TN https://orcid.org/0000-0001-6137-6114 Search for other works by this author on: This Site PubMed Google Scholar Anthony Merlocco, Anthony Merlocco 2Department of Diagnostic Imaging, St. Jude Children’s Research Hospital, Memphis, TN5Division of Pediatric Cardiology, University of Tennessee Health Science Center, Le Bonheur Children’s Hospital, Memphis, TN6Division of Pediatric Radiology, University of Tennessee Health Science Center, Le Bonheur Children’s Hospital, Memphis, TN https://orcid.org/0000-0001-6228-0229 Search for other works by this author on: This Site PubMed Google Scholar Chris Goode, Chris Goode 2Department of Diagnostic Imaging, St. Jude Children’s Research Hospital, Memphis, TN Search for other works by this author on: This Site PubMed Google Scholar Parul Rai, Parul Rai 7Department of Hematology, St. Jude Children’s Research Hospital, Memphis, TN https://orcid.org/0000-0002-5770-7280 Search for other works by this author on: This Site PubMed Google Scholar Jeffrey A. Towbin, Jeffrey A. Towbin 5Division of Pediatric Cardiology, University of Tennessee Health Science Center, Le Bonheur Children’s Hospital, Memphis, TN Search for other works by this author on: This Site PubMed Google Scholar Jane S. Hankins, Jane S. Hankins † 7Department of Hematology, St. Jude Children’s Research Hospital, Memphis, TN https://orcid.org/0000-0003-4439-7321 Search for other works by this author on: This Site PubMed Google Scholar Jason N. Johnson Jason N. Johnson † 2Department of Diagnostic Imaging, St. Jude Children’s Research Hospital, Memphis, TN5Division of Pediatric Cardiology, University of Tennessee Health Science Center, Le Bonheur Children’s Hospital, Memphis, TN6Division of Pediatric Radiology, University of Tennessee Health Science Center, Le Bonheur Children’s Hospital, Memphis, TN https://orcid.org/0000-0003-0712-1123 Search for other works by this author on: This Site PubMed Google Scholar Blood (2023) 141 (11): 1358–1362. https://doi.org/10.1182/blood.2022018209 Article history Submitted: August 26, 2022 Accepted: November 14, 2022 Connected Content This is a related article to: Heart failure in SCA: still challenging Share Icon Share Facebook Twitter LinkedIn MailTo Tools Icon Tools Request Permissions Cite Icon Cite Search Site Citation Cara E. Morin, Akshay Sharma, Subodh Selukar, Gary Beasley, Anthony Merlocco, Chris Goode, Parul Rai, Jeffrey A. Towbin, Jane S. Hankins, Jason N. Johnson; Diffuse myocardial fibrosis occurs in young patients with sickle cell anemia despite early disease-modifying therapy. Blood 2023; 141 (11): 1358–1362. doi: https://doi.org/10.1182/blood.2022018209 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search Search Dropdown Menu toolbar search search input Search input auto suggest filter your search All ContentAll JournalsBlood Search Subjects: Clinical Trials and Observations, Pediatric Hematology, Red Cells, Iron, and Erythropoiesis, Sickle Cell Disease, Transplantation TO THE EDITOR: We read with great interest the recent report from Niss et al.1 Our group has recently reviewed data from a similar population at our institution, which does not support the conclusion of Niss et al that diffuse myocardial fibrosis is prevented by early initiation of disease-modifying therapy (DMT). Cardiovascular complications are the leading cause of early mortality in patients with sickle cell anemia (SCA).2 Yet, the pathobiology of sickle cell cardiomyopathy is incompletely understood and likely multifactorial.3 Chronic anemia leads to global cardiac enlargement and myocardial hypertrophy. Global cardiac chamber dilation is a common feature in patients with SCA, and enlarged left atrial (LA) volume is a marker of diastolic dysfunction in SCA.4 Repetitive microvascular ischemic insults and associated reperfusion injury may contribute to diffuse myocardial fibrosis and myocardial remodeling in individuals with SCA....