Abstract
Purpose
The purpose of this study was to assess the role of various diagnostic tests in early detection of retinal changes in β-thalassemia major patients.
Methods
Thirty-eight visually asymptomatic β-thalassemia major patients receiving regular blood transfusions and iron-chelation therapy with deferoxamine (group A, n = 13), deferasirox (group B, n = 11) or deferoxamine with deferiprone (group C, n = 14) and fourteen age- and sex- matched healthy individuals were included in the study. All participants underwent ophthalmoscopy, full-field electroretinography (ERG), visual evoked potentials (VEP), multifocal electroretinography (mfERG), fundus autofluorescence (FAF) imaging and optical coherence tomography (OCT) scans.
Results
Retinal pigment epithelium changes were present in two cases. Scotopic ERG demonstrated decreased a-wave amplitude in groups A, B and C (p = 0.03, p = 0.002 and p = 0.002, respectively) and decreased b-wave amplitude in groups B and C (p = 0.002 and p = 0.01, respectively) compared to controls. Photopic ERG showed delayed b-wave latency in groups A and C (p = 0.03 and p = 0.03, respectively) ERG maximal combined response and VEP response did not differ between groups. MfERG showed reduced retinal response density in ring 1 in groups A, B, C (p < 0.001, p < 0.001, p = 0.001, respectively) and ring 2 in group B (p = 0.02) and delayed latency in ring 5 in groups A and B (p = 0.04 and p = 0.04, respectively). Abnormal FAF images appeared in three cases and OCT abnormalities in one case, whereas no changes were observed in controls (p = 0.55 and p = 1.00, respectively).
Conclusions
Full-field ERG and mfERG are more sensitive tools for detecting early retinal changes in β-thalassemia patients compared with ophthalmoscopy, VEP, FAF imaging and OCT scans.
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References
Galanello R, Origa R (2010) Beta-thalassemia. Orphanet J Rare Dis 5:11
Angelucci E, Brittenham GM, McLaren CE et al (2000) Hepatic iron concentration and total body iron stores in thalassemia major. N Engl J Med 343:327–31
Porter JB, Garbowski M (2014) The pathophysiology of transfusional iron overload. Hematol Oncol Clin North Am 28(683-701):vi
Borgna-Pignatti C, Marsella M (2015) Iron Chelation in Thalassemia Major. Clin Ther 37:2866–77
Bhoiwala DL, Dunaief JL (2016) Retinal abnormalities in beta-thalassemia major. Surv Ophthalmol 61:33–50
Di Nicola M, Barteselli G, Dell’Arti L et al (2015) Functional and Structural Abnormalities in Deferoxamine Retinopathy: A Review of the Literature. Biomed Res Int 2015:249617
Kertes PJ, Lee TK, Coupland SG (2004) The utility of multifocal electroretinography in monitoring drug toxicity: deferoxamine retinopathy. Can J Ophthalmol 39:656–61
Gelman R, Kiss S, Tsang SH (2014) Multimodal imaging in a case of deferoxamine-induced maculopathy. Retin Cases Brief Rep 8:306–9
Wu CH, Yang CP, Lai CC et al (2014) Deferoxamine retinopathy: spectral domain-optical coherence tomography findings. BMC Ophthalmol 14:88
Simon S, Athanasiov PA, Jain R et al (2012) Desferrioxamine-related ocular toxicity: a case report. Indian J Ophthalmol 60:315–7
Bansal V, Elgarbly I, Ghanchi FD, Atkinson PL (2003) Bull’s eye maculopathy with deferoxamine. Eur J Haematol 70:420–1
Genead MA, Fishman GA, Anastasakis A, Lindeman M (2010) Macular vitelliform lesion in desferrioxamine-related retinopathy. Doc Ophthalmol 121:161–6
McCulloch DL, Marmor MF, Brigell MG et al (2015) ISCEV Standard for full-field clinical electroretinography (2015 update). Doc Ophthalmol 130:1–12
Odom JV, Bach M, Brigell M et al (2010) ISCEV standard for clinical visual evoked potentials (2009 update). Doc Ophthalmol 120:111–9
Hood DC, Bach M, Brigell M et al (2012) ISCEV standard for clinical multifocal electroretinography (mfERG) (2011 edition). Doc Ophthalmol 124:1–13
Viola F, Barteselli G, Dell’arti L et al (2012) Abnormal fundus autofluorescence results of patients in long-term treatment with deferoxamine. Ophthalmology 119:1693–700
Hood DC, Seiple W, Holopigian K, Greenstein V (1997) A comparison of the components of the multifocal and full-field ERGs. Vis Neurosci 14:533–544
Arden GB, Wonke B, Kennedy C, Huehns ER (1984) Ocular changes in patients undergoing long-term desferrioxamine treatment. Br J Ophthalmol 68:873–7
Jiang C, Hansen RM, Gee BE et al (1998) Rod and rod mediated function in patients with beta-thalassemia major. Doc Ophthalmol 96:333–45
Marmor MF, Kellner U, Lai TY et al (2011) Revised recommendations on screening for chloroquine and hydroxychloroquine retinopathy. Ophthalmology 118:415–22
Ponjavic V, Andreasson S (2001) Multifocal ERG and full-field ERG in patients on long-term vigabatrin medication. Doc Ophthalmol 102:63–72
Ritter C, Renner AB, Wachtlin J et al (2008) Tamoxifen retinopathy: a case series of clinical and functional data. Ophthalmologe 105:544–9
Dettoraki M, Moschos MM (2016) The Role of Multifocal Electroretinography in the Assessment of Drug-Induced Retinopathy: A Review of the Literature. Ophthalmic Res 56:169–77
Schmidt D, Finke J (2004) Bull’s-Eye Maculopathy with Deferoxamine treatment. Klin Monbl Augenheilkd 221:204–9
El-Fattah El-Shazly AA, Ebeid WM, Elkitkat RS, Deghedy MR (2016) ELECTRORETINOGRAPHIC AND VISUAL-EVOKED POTENTIAL CHANGES IN RELATION TO CHELATION MODALITY IN CHILDREN WITH THALASSEMIA. Retina. doi:10.1097/IAE.0000000000001315
Koinzer S, Klettner A, Treumer F et al (2012) Correlation of fundus autofluorescence, spectral-domain optical coherence tomography, and microperimetry in late deferoxamine maculopathy. Retin Cases Brief Rep 6:50–5
El Shazly AA, Elkitkat RS, Ebeid WM, Deghedy MR (2016) CORRELATION BETWEEN SUBFOVEAL CHOROIDAL THICKNESS AND FOVEAL THICKNESS IN THALASSEMIC PATIENTS. Retina 36:1767–72
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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.
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Dettoraki, M., Kattamis, A., Ladas, I. et al. Electrophysiological assessment for early detection of retinal dysfunction in β-thalassemia major patients. Graefes Arch Clin Exp Ophthalmol 255, 1349–1358 (2017). https://doi.org/10.1007/s00417-017-3650-9
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DOI: https://doi.org/10.1007/s00417-017-3650-9