Sickle Cell Maculopathy: Prevalence, Associations and Impact on Visual Acuity

Sahak, Haroen; Saqalain, Mohammed; Lott, Pooi Wah; McKibbin, Martin

doi:10.1159/000512636

Cited by 6 publications

(7 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…2 shows the hazard ratios of HbSC to HbSS and HbSβ 0 thal for each of the individual disease-related chronic conditions. In keeping with previous reports 24 , 25 , compared to those with HbSS and HbSβ 0 thal, patients with HbSC were more likely to develop retinopathy ( P < 0.0001). However, their risks for chronic kidney disease ( P = 0.014), pulmonary hypertension ( P = 0.0075) and chronic leg ulcers ( P = 0.001) were lower.…”

Section: Resultssupporting

confidence: 91%

Hematopoietic Stem Cell Transplant for Sickle Cell Disease: PATIENT SELEction and Timing Based on Sickle Cell-Related Multiple Chronic Conditions

Jang

Stewart

et al. 2021

Cell Transplant

View full text Add to dashboard Cite

Hematopoietic stem cell transplant (HSCT) is the only cure for patients with sickle cell disease (SCD). Although most SCD patients experience progressive end-organ damage and shortened lifespans, not all patients follow the same disease course, tempo, or outcome. Therefore, the dilemma facing physicians is weighing the selection of patients and timing for the procedure against donor type and transplant-related mortality and morbidity that go up with increasing age. On the other hand, the dilemma facing the patients and families is how acceptable HSCT that carries some mortality risks to them. We have analyzed the chronic conditions due to SCD in 449 patients to determine whether SCD-related multiple chronic conditions (MCC) can be risk-stratified to identify the group of patients predicted to not only have shortened lifespans but also functional limitation and poor quality of life so that these at-risk patients can be offered HSCT early and before MCC develops. We identified that the age of onset of the first SCD-related chronic conditions strongly predicted for the risks for disease-related MCC. SCD patients who suffered their first disease-related chronic condition before age 30 years developed MCC at a rate of 19.1 times faster than those at a later age. These patients are therefore high-risk patients and should be offered HSCT early in the course of their disease before multiple organ damage intervenes, even if matched-related donors are not available. This patient selection and timing approach provides a forum for an easy-to-understand and real-time discussion, including the choice of donor type, with SCD patients and families when considering HSCT.

show abstract

Section: Resultssupporting

confidence: 91%

Hematopoietic Stem Cell Transplant for Sickle Cell Disease: PATIENT SELEction and Timing Based on Sickle Cell-Related Multiple Chronic Conditions

Jang

Stewart

et al. 2021

Cell Transplant

View full text Add to dashboard Cite

show abstract

“…This frequency is in accordance with previous values that exhibit large variation from 53.6% to 29%. 7,11,17 None of our patients had a symptomatic visual impairment. 4 However, different authors reported asymptomatic involvement of the visual field and contrast sensibility.…”

Section: Discussionmentioning

confidence: 81%

“…But, when tested, patients with SCD with such abnormalities exhibit decrease in retinal sensitivity, visual field defects, and perturbation of contrast sensitivity. 10,11 Several questions regarding this macular or parafoveal involvement are still unsolved. It has been suggested that they could share the same mechanism as silent border zone cerebral infarct.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Atypical Foveal and Parfoveal Abnormalities in Sickle Cell Disease

Orssaud,

Flammarion,

Michon

et al. 2023

Retina

View full text Add to dashboard Cite

Purpose: The primary aim was to describe patterns of para macular involvement, not yet reported but that OCT-A can now detect in SCD patients. The secondary aim was to search arguments concerning the physio pathogeny of para macular involvement. Methods: This institutional cohort retrospective study was conducted in a Referral Center for Ophthalmological Rare Diseases. Follow-up included an ophthalmological examination with optical coherent tomography and optical coherent tomography angiography. Results: Hundred and thirty-two sickle cell patients were included. Typical sickle cell maculopathy was observed in temporal area in 84 eyes (40.0 %) of SS patients and 8 eyes (14.8 %) of SC patients (p < 0.001). Enlargement of the foveal avascular zone was observed in 10 eyes whom 8 of SS patients. Two atypical parafoveal abnormalities were found in SS patients only. The first one consisted in macular thinning with normal vascularization in 15 eyes of 11 patients. The second atypical maculopathy was large areas of loss of vascularization without retina thinning 10 eyes of six patients. Multivariate analysis did not show a statistically significant relation between the PSR stage and the different type of sickle cell maculopathy (p = 0.21) Conclusion: Those atypical sickle cell maculopathy may correspond to early forms preceding a typical SCM. This would point towards several physiopathogenic mechanisms. The first one included the existence of ischemia which can be related to anemia. Presence of retinal thinning without vascular involvement point out to a neurogenic mechanism.

show abstract

“…Factors examined for an association with the development of SCR and PSCR included: gender, hemoglobin genotype, blood pressure (BP), body mass index (BMI), the presence of systemic SCD complications (e.g., seizure, cerebrovascular accident [CVA], hematuria, nephrotic syndrome, hearing loss, heart disease, hand foot syndrome, spleen infarction, pneumonia, lung infarction, leg ulcers, or previous painful crisis), smoking history (including the use of cigarettes, cigars, and/or pipes) and hematological laboratory testing (e.g., WBC, platelets, and reticulocyte counts, hemoglobin, and HbF levels) [10,[17][18][19][20][21][22]. BP was considered to be abnormal if systolic pressure was ≥ 130 mmHg or diastolic pressure was ≥ 90 mmHg.…”

Section: Methodsmentioning

confidence: 99%

Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease

et al. 2022

View full text Add to dashboard Cite

Background Sickle cell retinopathy (SCR) is one of the most important ocular manifestations of sickle cell disease (SCD). This study aims to assess the prevalence of SCR in SCD, identify risk factors for its development and progression to proliferative sickle cell retinopathy (PSCR), and evaluate the potential implications of these results on clinical practice. Methods This research is a secondary analysis of patients diagnosed with SCD from the epidemiological, multicenter Cooperative Study of Sickle Cell Disease (CSSCD). We included all patients who completed a full ophthalmic evaluation. We identified clinical and laboratory SCD characteristics associated with SCR using multivariate logistic regression models. Proliferative sickle cell retinopathy (PSCR) was diagnosed according to the Goldberg classification system. Results Of the 1904 study participants with SCD who met the inclusion criteria, 953 (50.1%) had retinopathy; of which 642 (67.3%) had bilateral disease. SCR was associated with older age (p < 0.001), history of smoking (p = 0.001), hematuria (p = 0.050), and a lower hemoglobin F (HbF) level (p < 0.001). PSCR risk increased with smoking (p = 0.005), older age (p < 0.001) higher hemoglobin level (p < 0.001) and higher white blood cell count (p = 0.011). Previous blood transfusion (p = 0.050), higher reticulocyte count (p = 0.019) and higher HbF level (p < 0.001) were protective factors against the development of PSCR. Ocular symptoms were associated with progression to PSCR in patients with SCR (p = 0.021). Conclusion In this cohort of individuals with SCD, half of the participants had signs of SCR. Smoking and blood hemoglobin level were the two modifiable risk factors associated with increased retinopathy progression. Screening to identify the different stages of retinopathy, actively promoting smoking cessation, and optimizing the hematological profile of patients with SCD should guide treatment protocols designed to prevent the vision-threatening complications of the disease.

show abstract

Sickle Cell Maculopathy: Prevalence, Associations and Impact on Visual Acuity

Cited by 6 publications

References 25 publications

Hematopoietic Stem Cell Transplant for Sickle Cell Disease: PATIENT SELEction and Timing Based on Sickle Cell-Related Multiple Chronic Conditions

Hematopoietic Stem Cell Transplant for Sickle Cell Disease: PATIENT SELEction and Timing Based on Sickle Cell-Related Multiple Chronic Conditions

Atypical Foveal and Parfoveal Abnormalities in Sickle Cell Disease

Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease

Contact Info

Product

Resources

About