June 2020
Volume 61, Issue 7
Free
ARVO Annual Meeting Abstract  |   June 2020
Hematologic and Systemic Risk Factors for Progression of Sickle Cell Retinopathy
Author Affiliations & Notes
  • Kevin Dahlan
    Albert Einstein College of Medicine, Bronx, New York, United States
  • Michelle Goodman
    Albert Einstein College of Medicine, Bronx, New York, United States
  • Kripali Kripali Gautam
    Albert Einstein College of Medicine, Bronx, New York, United States
  • Pamela D Suman
    Ophthalmology, Montefiore Medical Center, New York, United States
  • Caterina Minniti
    Hematology, Montefiore Medical Center, New York, United States
    Albert Einstein College of Medicine, Bronx, New York, United States
  • umar mian
    Ophthalmology, Montefiore Medical Center, New York, United States
    Albert Einstein College of Medicine, Bronx, New York, United States
  • Footnotes
    Commercial Relationships   Kevin Dahlan, None; Michelle Goodman, None; Kripali Kripali Gautam, None; Pamela Suman, None; Caterina Minniti, None; umar mian, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2020, Vol.61, 1307. doi:
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    • Get Citation

      Kevin Dahlan, Michelle Goodman, Kripali Kripali Gautam, Pamela D Suman, Caterina Minniti, umar mian; Hematologic and Systemic Risk Factors for Progression of Sickle Cell Retinopathy. Invest. Ophthalmol. Vis. Sci. 2020;61(7):1307.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : There are currently no established guidelines for disease monitoring for sickle cell retinopathy (PSR) in patients with sickle cell disease. Sickle cell comorbidities and hematologic laboratory indices may be useful metrics to identify patients at higher risk for PSR progression. This study examines the association between PSR progression and sickle cell comorbidities, healthcare utilization, and hematologic laboratory values.

Methods : A single-institution case-controlled study was conducted identifying adult sickle cell patients receiving care at Montefiore Medical Center. Inclusion criteria included patients of any sickle cell genotype who had at least 2 fluorescein angiograms (FA) in the past 5 years. Fluorescein angiograms were reviewed by a retina ophthalmologist for disease progression, defined by new neovascularization not seen on prior imaging. The electronic medical record was used to gather information about laboratory values, emergency room (ED) visits, hospitalizations, and comorbidities. Univariate logistic regression was used to determine associations of these metrics and progression of disease.

Results : A total of 50 patients were included in this study with an average age of 41. Twenty-seven patients (54%) had Hb-SS disease, 22 (44%) had Hb-SC disease, and 1 (2%) had SO Arab. Patients had an average of 2.84 angiograms taken within an average span of 2.07 years. Seven patients (14%) demonstrated progression of PSR while 43 patients (86%) did not. Patients demonstrating PSR progression had a lower hemoglobin F (p=.04), a higher prevalence of acute chest syndrome (p=.04), more ED visits (p=.003) and hospitalizations (p=.0008) than patients without PSR progression (OR=0.988 ± .011; 1.22 ± .21; 1.02 ± .01; 1.05 ± .03). Genotype, other comorbidities, and laboratory indices did not demonstrate significant association.

Conclusions : Our results demonstrate that PSR progression is associated with a low hemoglobin F, a higher level of inpatient healthcare utilization, and acute chest syndrome. This may indicate that patients with lower Hb F, a prior diagnosis of acute chest syndrome or frequent hospitalizations and ED visits may benefit from more frequent ophthalmology evaluations to assess for PSR progression. Further studies should prospectively evaluate if patients with acute chest syndrome or low levels of Hb F develop progression of PSR at a higher rate.

This is a 2020 ARVO Annual Meeting abstract.

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