As the predominant cell type in blood, red blood cells (RBCs) and their biomechanical properties largely determine the rheological and hemodynamic behavior of blood in normal and disease states. In sickle cell disease (SCD), mechanically fragile, poorly deformable RBCs contribute to impaired blood flow and other pathophysiological aspects of the disease. The major underlying cause of this altered blood rheology and hemodynamics is hemoglobin S (HbS) polymerization and RBC sickling under deoxygenated conditions. This review discusses the characterization of the biomechanical properties of sickle RBCs and sickle blood as well as their implications toward a better understanding of the pathophysiology of the disease.
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http://dx.doi.org/10.1146/annurev-bioeng-070909-105339 | DOI Listing |
Womens Health Rep (New Rochelle)
December 2024
National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, USA.
Although there is an emerging body of evidence that addresses the adverse health outcomes of individuals with sickle cell trait (SCT), it is not clear if the findings are generalizable from a sex and gender perspective. The purpose of this scoping review was to complete an assessment of main concepts, identify knowledge gaps, and determine the potential value of conducting an eventual systematic review. The research question guiding this scoping review is: In SCT individuals with adverse health outcomes, what is known about sex and gender differences? We conducted a scoping review of research on SCT from 2016 to 2022 across five databases, with 36 research studies included in the review.
View Article and Find Full Text PDFActa Biochim Biophys Sin (Shanghai)
January 2025
Innovation Center for Diagnostics and Treatment of Thalassemia, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China.
Reactivating the embryonic ζ-globin gene represents a potential therapeutic approach to ameliorate the severe clinical phenotype of α-thalassemia and sickle cell disease. The transcription factor MYB has been extensively proven to be a master regulator of the γ-globin gene, but its role in the regulation of ζ-globin remains incompletely understood. Here, we report a mechanistic study on the derepression of ζ-globin both and .
View Article and Find Full Text PDFLancet Haematol
January 2025
St Jude Children's Research Hospital, Memphis, TN 38107, USA. Electronic address:
Am J Hematol
January 2025
Byers Eye Institute at Stanford University School of Medicine, Watson, Palo Alto, California, US.
Indian J Pediatr
January 2025
Department of Biochemistry, All India Institute of Medical Sciences, Bibinagar, Hyderabad, Telangana, India.
This hospital-based cross-sectional study aimed to screen newborns for sickle cell anemia immediately after birth and validate dried blood spot (DBS) samples against conventional venous blood samples (CBS) for hemoglobin variant analysis by HPLC. Among 751 newborns, 2.93% were found to have sickle cell trait.
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