Orssaud Christophe, Flamarion Edouard, Michon Adrien, Ranque Brigitte, Arlet Jean Benoit
Functional Unity of Ophthalmology, ERN EYE, Ophthalmological Rare Diseases Center, Georges Pompidou European Hospital, Assistance Publique-Hopitaux de Paris, Paris, France.
Internal Medicine Department, French National Sickle Cell Referral Center, European Hospital Georges Pompidou, Paris, France.
Front Med (Lausanne). 2023 Aug 28;10:1226210. doi: 10.3389/fmed.2023.1226210. eCollection 2023.
To identify risk factors for sickle cell maculopathy due to hematological parameters (especially anemia and hemolysis) or cerebral vasculopathy.
This retrospective study was conducted at a Referral Center. The follow-up included optical coherent tomographyoptical coherent tomography angiography, neuro-radiological imaging, and a hematological assessment hemoglobin, hemoglobin S level, reticulocytes, mean corpuscular volume, bilirubin, and lactate dehydrogenase).
Hundred and thirty-two sickle cell patients were included. Maculopathy was observed in 127 eyes of SS patients and 10 eyes of SC patients ( < 0.001), unrelated to peripheral retinopathy. Cerebral vasculopathy was more frequent in SS patients ( < 0.001) and was also associated with the presence of maculopathy ( = 0.049), and it was related to peripheral retinopathy ( < 0.001). All biological parameters significantly differed according to the genotype ( < 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.
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.
确定因血液学参数（尤其是贫血和溶血）或脑血管病变导致镰状细胞性黄斑病变的危险因素。
本回顾性研究在一家转诊中心进行。随访包括光学相干断层扫描、光学相干断层扫描血管造影、神经放射学成像以及血液学评估（血红蛋白、血红蛋白S水平、网织红细胞、平均红细胞体积、胆红素和乳酸脱氢酶）。
纳入了132例镰状细胞病患者。在SS患者的127只眼中和SC患者的10只眼中观察到黄斑病变（P<0.001），与周边视网膜病变无关。脑血管病变在SS患者中更常见（P<0.001），并且也与黄斑病变的存在相关（P = 0.049），且与周边视网膜病变有关（P<0.001）。所有生物学参数根据基因型有显著差异（P<0.001），但根据脑血管病变或黄斑病变的存在无差异。在多变量分析中，网织红细胞和胆红素与脑血管病变和黄斑病变的存在相关。
获得的数据与贫血或溶血标志物在脑血管病变和黄斑受累中的作用一致。由于溶血趋势似乎是这些并发症的一个危险因素，这证实了在这些患者中使用预防性血浆置换的合理性。
