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. 2023 Aug 28:10:1226210.
doi: 10.3389/fmed.2023.1226210. eCollection 2023.

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

Christophe Orssaud  1 , Edouard Flamarion  2 , Adrien Michon  2 , Brigitte Ranque  2   3 , Jean Benoit Arlet  2   4   5   6
Affiliations

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

Christophe Orssaud et al. Front Med (Lausanne). .

Abstract

Purpose: To identify risk factors for sickle cell maculopathy due to hematological parameters (especially anemia and hemolysis) or cerebral vasculopathy.

Methods: This retrospective study was conducted at a Referral Center. The follow-up included optical coherent tomography/optical coherent tomography angiography, neuro-radiological imaging, and a hematological assessment (hemoglobin, hemoglobin S level, reticulocytes, mean corpuscular volume, bilirubin, and lactate dehydrogenase).

Results: Hundred and thirty-two sickle cell patients were included. Maculopathy was observed in 127 eyes of SS patients and 10 eyes of SC patients (p < 0.001), unrelated to peripheral retinopathy. Cerebral vasculopathy was more frequent in SS patients (p < 0.001) and was also associated with the presence of maculopathy (p = 0.049), and it was related to peripheral retinopathy (p < 0.001). All biological parameters significantly differed according to the genotype (p < 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.

Conclusion: 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.

Keywords: OCT; cerebral vasculopathy; hemolysis (red blood cells); maculopathy; optical coherence tomography angiography (OCT-A); sickle cell disease.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Parafoveolar retinal thinning and reduction of capillaries in the SCP and DCP. (A) Reduction of the capillaries in the SCP; (B) Reduction of the capillaries in the DCP; (C) Retinal thinning of the inner retinal layers (blue arrow). SCP, superficial capillary plexuses; DCP, deep capillary plexuses.
Figure 2
Figure 2
Small parafoveolar retinal thinning and reduction of capillaries in the SCP and DCP. (A) Reduction of capillaries in the SCP; (B) Reduction of capillaries in the DCP; (C) Retinal thinning of the inner retinal layers (blue arrow); and (D) Inferotemporal retinal thinning (blue area on the retinal thickness map). SCP, superficial capillary plexuses; DCP, deep capillary plexuses.
See this image and copyright information in PMC

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