The role of red blood cell characteristics and viscosity in sickle cell retinopathy and maculopathy.

Details

Ressource 1Download: 40296702_BIB_A2D04943F314.pdf (1171.82 [Ko])
State: Public
Version: Final published version
License: CC BY-NC 4.0
Serval ID
serval:BIB_A2D04943F314
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
The role of red blood cell characteristics and viscosity in sickle cell retinopathy and maculopathy.
Journal
British journal of haematology
Author(s)
Brandsen R.P., Diederen RMH, Klaassen I., Veldthuis M., Korsten H., van Zwieten R., Schlingemann R.O., Nur E., Biemond B.J.
ISSN
1365-2141 (Electronic)
ISSN-L
0007-1048
Publication state
Published
Issued date
06/2025
Peer-reviewed
Oui
Volume
206
Number
6
Pages
1796-1805
Language
english
Notes
Publication types: Journal Article
Publication Status: ppublish
Abstract
Sickle cell disease (SCD), encompassing genotypes such as HbSS and HbSC, causes chronic haemolysis and microvascular occlusion, leading to organ damage. The retina is particularly vulnerable, often resulting in sickle cell retinopathy (SCR) or sickle cell maculopathy (SCM). The precise underlying mechanisms are unclear, though various factors are suggested to contribute. This study explored the role of whole blood viscosity and red blood cell (RBC) deformability in SCR and SCM. Adult HbSS (n = 34) and HbSC patients (n = 34) were offered an ophthalmic examination to determine SCR stage. A venous ethylenediaminetetraacetic acid (EDTA) sample was collected from each participant. Whole blood viscosity was measured using a Brookfield viscometer and RBC deformability was assessed using the Oxygenscan feature of the Laser Optical Rotational Red Cell Analyser as a function of the (varying) partial oxygen pressure. HbSC patients with proliferative sickle cell retinopathy (PSCR) had a lower delta elongation index (p = 0.012) and point of sickling (p = 0.002) than those without PSCR, suggesting that RBC sickling might not play a central role in the pathogenesis of PSCR in HbSC patients. Despite hyperviscosity being a commonly proposed mechanism, no associations were found between blood viscosity, SCR and SCM. These results point to alternative mechanisms contributing to SCR and SCM, highlighting the complexity and need for further research to fully understand the underlying factors.
Keywords
Humans, Anemia, Sickle Cell/blood, Anemia, Sickle Cell/complications, Blood Viscosity, Retinal Diseases/blood, Retinal Diseases/etiology, Adult, Female, Male, Erythrocyte Deformability, Erythrocytes/pathology, Middle Aged, Young Adult, erythrocyte deformability, sickle cell disease, sickle cell maculopathy, sickle cell retinopathy, viscosity
Pubmed
Web of science
Open Access
Yes
Create date
12/05/2025 8:42
Last modification date
08/07/2025 7:16
Usage data