Abstract
Purpose :
Hematopoietic stem cell transplantation (HSCT) for sickle cell disease (SCD) has enabled the conversion of hemoglobin SS genotype to either normal hemoglobin AA or to sickle cell trait (Hgb AS) with cessation of systemic crises. While peripheral vasculature abnormalities are the hallmark of sickle cell retinopathy (SCR), prior work by our group and others has shown maculopathy (SCM) characterized as macular thinning and vascular abnormalities on spectral-domain optical coherence tomography (SDOCT) and optical coherence tomography angiography (OCTA). The purpose of this study was to determine whether HSCT resulted in any change in the level of SCR or rate of SCM thinning.
Methods :
A retrospective, cross-sectional chart review was performed. Post HSCT SCD patients were identified through a comprehensive chart review of the prospective SCR Study patient database. The SCR Study patients all underwent an annual complete ophthalmic examination, macular SDOCT, and OCTA imaging. A multivariate logistic regression analysis was performed to analyze the relationship between SDOCT thickness and patient transplantation status.
Results :
A total of thirteen patients were followed longitudinally over a study interval of ten years and compared to those without HSCT. Best-corrected visual acuity was preserved over the study interval (Pre-HSCT: LogMAR: 0.0821, Post-HSCT: LogMAR: 0.0878, p=0.0023). Average macular thickness prior to HSCT was (312.94 +/- 16.88 μm) and post HSCT was (308.49 +/- 21.911 μm). Despite HSCT, study patients continued to demonstrate a generalized trend for continued macular thinning at rates similar to pre-transplantation (pre-HSCT: -0.276 μm/year; post-HSCT: -0.321 μm/year, p=0.572). No strong associations between SDOCT thickness/rates of thinning and parameters such as Hgb type, smoking status, hydroxyurea use, number of pain crises were identified for the HSCT subgroup.
Conclusions :
Progression of SCM appears to occur despite HSCT. Further study using other imaging modalities such as OCTA may provide additional insight into the biological and pathophysiological mechanisms of SCM in HSCT patients.
This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.