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Objectives: Allogeneic haematopoietic stem cell transplantation (allo-HSCT) is the only currently available curative treatment for sickle cell disease (SCD). Here, we comprehensively evaluated the reconstitution of T- and B-cell compartments in 29 SCD patients treated with allo-HSCT and how it correlated with the development of acute graft-versus-host disease (aGvHD).
Methods: T-cell neogenesis was assessed by quantification of signal-joint and β-chain TCR excision circles. B-cell neogenesis was evaluated by quantification of signal-joint and coding-joint K-chain recombination excision circles. T- and B-cell peripheral subset numbers were assessed by flow cytometry.
Results: Before allo-HSCT (baseline), T-cell neogenesis was normal in SCD patients compared with age-, gender- and ethnicity-matched healthy controls. Following allo-HSCT, T-cell neogenesis declined but was fully restored to healthy control levels at one year post-transplantation. Peripheral T-cell subset counts were fully restored only at 24 months post-transplantation. Occurrence of acute graft-versus-host disease (aGvHD) transiently affected T- and B-cell neogenesis and overall reconstitution of T- and B-cell peripheral subsets. B-cell neogenesis was significantly higher in SCD patients at baseline than in healthy controls, remaining high throughout the follow-up after allo-HSCT. Notably, after transplantation SCD patients showed increased frequencies of IL-10-producing B-regulatory cells and IgM memory B-cell subsets compared with baseline levels and with healthy controls.
Conclusion: Our findings revealed that the T- and B-cell compartments were normally reconstituted in SCD patients after allo-HSCT. In addition, the increase of IL-10-producing B-regulatory cells may contribute to improve immune regulation and homeostasis after transplantation.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9035210 | PMC |
http://dx.doi.org/10.1002/cti2.1389 | DOI Listing |
Radiol Case Rep
February 2025
Cardiology Department, University Hospital Agadir, Medical School of Medicine & Pharmacy Ibn Zohr University, Agadir, Morocco.
An anomalous origin of the right coronary artery from the opposite sinus (R- ACAOS) with interarterial course is a very rare congenital anomaly with an increased risk of sudden cardiac death. A 29-year-old woman was admitted for exertional angina pectoris. A coronary computed tomography angiography with 3D multiplanar reconstruction revealed an R-ACAOS running between the aorta and pulmonary artery with high anatomical features and no ischemia-induced at the stress test.
View Article and Find Full Text PDFG Ital Cardiol (Rome)
January 2025
U.O.C. Cardiologia 1, Dipartimento Cardiovascolare, ASST Papa Giovanni XXIII, Bergamo.
Mineralocorticoid receptor antagonists (MRAs) represent one of the cornerstones of treatment for heart failure with reduced ejection fraction. Post-hoc data from the TOPCAT trial, conducted in patients with heart failure mildly reduced or preserved ejection fraction (HFmrEF/HFpEF), suggest the possible clinical benefit of MRAs, particularly for slightly reduced ejection fraction values. The advent of non-steroidal MRAs, including finerenone, seems to represent a turning point in the treatment for HFmrEF/HFpEF.
View Article and Find Full Text PDFGlob Heart
December 2024
Instituto do Coração, Faculdade de Medicina Universidade de São Paulo, São Paulo, Brazil.
Background: Sudden cardiac death (SCD) is a major concern in patients with hypertrophic cardiomyopathy (HCM). The American College of Cardiology/American Heart Association (ACC/AHA) and the European Society of Cardiology (ESC) have different guidelines for SCD risk stratification. Their comparative performance in diverse populations remains uncertain.
View Article and Find Full Text PDFHealth Care Transit
January 2024
Hemoglobinopathy Clinic, Division of Haematology/Oncology, The Hospital for Sick Children, 555 University Ave., Toronto, ON, M5G 1X8, Canada.
Background: The transfer from paediatric to adult care presents a significant challenge for adolescents and young adults (AYA) with sickle cell disease (SCD). Disease self-management skills have been recognized as important mediators of poor health outcomes, but transition-related skills such as scheduling appointments and understanding the shift in health care responsibilities remain under taught in the healthcare system. The purpose of this quality improvement (QI) study was to (1) understand the areas of disease self-management that AYA patients felt underprepared for, and (2) design and evaluate an educational program addressing the top unmet skills.
View Article and Find Full Text PDFHealth Care Transit
March 2024
Boston University Chobanian & Avedisian School of Medicine, Boston, MA.
Background: Transition in sickle cell disease (SCD) is associated with an alarming increase in acute care utilization, cost, and risk of early mortality. Effective transition preparation is crucial to address these issues. We established a multidisciplinary transition clinic at our urban SCD center in the fall of 2021.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!