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Increased erythrocyte phosphatidylserine exposure in sickle cell disease: flow-cytometric measurement and clinical associations
Open accessAbstract
Increased exposure of phosphatidylserine (PS) in erythrocytes has been postulated to contribute to the pathophysiology of sickle cell disease because of its possible effects on blood coagulation, cell adhesion, and cell clearance. We developed a flow-cytometric assay to measure exposure of PS on the outer face of the erythrocyte membrane based on addition of fluorescein-annexin V to whole-blood specimens. The assay correlated linearly with binding of 125I-annexin V (r2 = .95, n = 125 samples). Normal donors (n = 30) showed virtually no annexin-positive cells (0.34% +/- 0.18% for 24-hour old samples). In contrast, annexin V binding was above the upper limit of normal in 96% of 205 specimens from 17 adult sickle cell and 2 beta-thalassemia patients; the mean percentage of annexin-positive cells was 2.86% +/- 2.00% (range, 0.4% to 12.0%). Values varied substantially over time for some patients, and mean values varied between patients. The percentage of annexin-positive cells always decreased after transfusion (11 events in 6 patients), and out of proportion to the amount of blood transfused. In conclusion, increased exposure of PS on a subpopulation of erythrocytes in vivo is a virtually universal feature of sickle cell disease, and its measurement may be useful to evaluate clinical status and response to therapeutic measures such as blood transfusion.
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“Notably, PS exposure on RBCs is increased in SCD (195).”
From Red Blood Cell Function and Dysfunction: Redox Regulation, Nitric Oxide Metabolism, Anemia (2017) · cites this paper as [195]
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Metadata
- Authors
- Wood, B., Gibson, D., & Tait, J.
- Affiliations
- University of Washington (US)
- Department of Laboratory Medicine, University of Washington, Seattle 98195–7110, USA.
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 1996-09-01
- Volume
- 88
- Issue
- 5
- Pages
- 1873-1880
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- OpenAlex
- W13114315
- Cited by
- 445
- References
- 34
- Citations by year
- 2026: 3 · 2025: 13 · 2024: 2 · 2023: 10 · 2022: 5 · 2021: 10 · 2020: 4 · 2019: 2 · 2018: 10 · 2017: 17 · 2016: 33 · 2015: 41 · 2014: 28 · 2013: 31 · 2012: 24
- FWCI
- 7.33
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Blood (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (5p50hl047151-05)
- National Heart, Lung, and Blood Institute (HL-47151)
- Topics
- Health Sciences › Medicine › Genetics (clinical) › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Physiology (medical) › Erythrocyte Function and Pathophysiology
- Health Sciences › Medicine › Hematology › Blood groups and transfusion
- Keywords
- sickle cell disease, phosphatidylserine exposure, erythrocytes, annexin V, beta-thalassemia, blood transfusion, flow cytometry assay, cell adhesion, coagulation, cell clearance
- MeSH terms
- Adult, Anemia, Sickle Cell, Erythrocyte Membrane, Erythrocytes, Abnormal, Flow Cytometry, Humans, Membrane Lipids, Phosphatidylserines, beta-Thalassemia, Annexin A5
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