AUTHOR=Orssaud Christophe , Flamarion Edouard , Michon Adrien , Ranque Brigitte , Arlet Jean Benoit 

TITLE=Relationship between paramacular thinning, cerebral vasculopathy, and hematological risk factors in sickle cell disease

JOURNAL=Frontiers in Medicine

VOLUME=10

YEAR=2023

URL=https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2023.1226210

DOI=10.3389/fmed.2023.1226210

ISSN=2296-858X

ABSTRACT=<sec><title>Purpose</title><p>To identify risk factors for sickle cell maculopathy due to hematological parameters (especially anemia and hemolysis) or cerebral vasculopathy.</p></sec><sec><title>Methods</title><p>This retrospective study was conducted at a Referral Center. The follow-up included optical coherent tomography<bold>/</bold>optical coherent tomography angiography, neuro-radiological imaging, and a hematological assessment <bold>(</bold>hemoglobin, hemoglobin S level, reticulocytes, mean corpuscular volume, bilirubin, and lactate dehydrogenase).</p></sec><sec><title>Results</title><p>Hundred and thirty-two sickle cell patients were included. Maculopathy was observed in 127 eyes of SS patients and 10 eyes of SC patients (<italic>p</italic> &lt; 0.001), unrelated to peripheral retinopathy. Cerebral vasculopathy was more frequent in SS patients (<italic>p</italic> &lt; 0.001) and was also associated with the presence of maculopathy (<italic>p</italic> = 0.049), and it was related to peripheral retinopathy (<italic>p</italic> &lt; 0.001). All biological parameters significantly differed according to the genotype (<italic>p</italic> &lt; 0.001) but not according to the presence of cerebral vasculopathy or maculopathy. In the multivariate analysis, reticulocytes and bilirubin were associated with the presence of cerebral vasculopathy and maculopathy.</p></sec><sec><title>Conclusion</title><p>The data obtained were consistent with the role of anemia or hemolysis markers in cerebral vasculopathy and macular involvement. As a trend of hemolysis appears to be a risk factor for these complications, this validates the use of preventive plasmapheresis in these patients.</p></sec>