Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress 2023 EHA Congress 2023 ASCO Annual Meeting
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on sleep in brain health

LIVE: Thursday 2nd May 2024, 18:00-19:30 (CEST)

Quality sleep is essential for health. But what happens to our brains when sleep patterns are disturbed? Join our experts to explore the interplay between sleep disruption and neurological diseases, and the questions that you need to be asking your patients to help you prevent the harmful effects of sleep deprivation.
ADVANCED SEARCH
Log in
Top
BMC Ophthalmology
Published in: BMC Ophthalmology 1/2018

Open Access 01-12-2018 | Case report

Pattern dystrophies in patients treated with deferoxamine: report of two cases and review of the literature

Authors: Constantine D. Georgakopoulos, Foteini Tsapardoni, Elli V. Kostopoulou, Olga E. Makri

Published in: BMC Ophthalmology | Issue 1/2018

Login to get access

Abstract

Background

Deferoxamine (DFO) is one of the most commonly used chelation treatments for transfusional hemosiderosis. Pattern dystrophies constitute a distinct entity of retinal disorders that has been occasionally identified in association with deferoxamine.

Case presentation

We report two cases of bilateral macular pattern dystrophy in transfusion dependent patients undergoing chronic chelation therapy with deferoxamine due to thalassemias. Our patients were evaluated with multimodal imaging and the results are presented. Both patients had normal cone and rod responses in the full-field electroretinogram and continued the prescribed chelation therapy, after hematology consult. The patients were followed up every 3 months for 2 and 4 years respectively for possible deterioration. Their best corrected visual acuity remained stable with no anatomic change on Optical Coherence Tomography findings.

Conclusion

Multimodal imaging of our patients allowed a better evaluation and possibly earlier detection of the DFO-related changes. Screening and close follow up of patients under chronic chelating therapy is important in order to promptly diagnose and manage possible toxicity either with discontinuation of the offending agent or dose modification.
Literature
1.
2.
Lakhanpal V, Schocket SS, Jiji R. Deferoxamine (Desferal)-induced toxic retinal pigmentary degeneration and presumed optic neuropathy. Ophthalmology. 1984;91:443–51.CrossRefPubMed
3.
Haimovici R, D’Amico DJ, Gragoudas ES, Sokol S. The expanded clinical spectrum of deferoxamine retinopathy. Ophthalmology. 2002;109:164–71.CrossRefPubMed
4.
Marmor MF, Fulton AB, Holder GE, Miyake Y, Brigell M, Bach M. ISCEV standard for full-field clinical electroretinography (2008 update). Doc Ophthalmol. 2009;118:69–77.CrossRefPubMed
5.
Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH, Jordan L, Lanzkron SM, Lottenberg R, Savage WJ, Tanabe PJ, Ware RE, Murad MH, Goldsmith JC, Ortiz E, Fulwood R, Horton A, John-Sowah J. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014;312:1033–48.CrossRefPubMed
6.
Taher A, Bashshur Z, Shamseddeen WA, Abdulnour RE, Aoun E, Koussa S, Baz P. Ocular findings among thalassemia patients. Am J Ophthalmol. 2006;142:704–5.CrossRefPubMed
7.
Taneja R, Malik P, Sharma M, Agarwal MC. Multiple transfused thalassemia major: ocular manifestations in a hospital-based population. Indian J Ophthalmol. 2010;58:125–30.CrossRefPubMedPubMedCentral
8.
De Virgiliis S, Congia M, Turco MP, Frau F, Dessi C, Argiolu F, Sorcinelli R, Sitzia A, Cao A. Depletion of trace elements and acute ocular toxicity induced by desferrioxamine in patients with thalassaemia. Arch Dis Child. 1988;63(3):250–5.CrossRefPubMedPubMedCentral
9.
Arora A, Wren S, Gregory Evans K. Desferrioxamine related maculopathy: a case report. Am J Hematol. 2004;76:386–8.CrossRefPubMed
10.
Aessopos A, Savvides P, Stamatelos G, et al. Pseudoxanthoma elasticum-like skin lesions and angioid streaks in beta-thalassemia. Am J Hematol. 1992;41:159–64.CrossRefPubMed
11.
Barteselli G, Dell'arti L, Finger RP, Charbel Issa P, Marcon A, Vezzola D, Mapelli C, Cassinerio E, Cappellini MD, Ratiglia R, Viola F. The spectrum of ocular alterations in patients with beta-thalassemia syndromes suggests a pathology similar to pseudoxanthoma elasticum. Ophthalmology. 2014;121:709–18.CrossRefPubMed
12.
Aessopos A, Voskaridou E, Kavouklis E, Vassilopoulos G, Rombos Y, Gavriel L, Loukopoulos D. Angioid streaks in sickle-thalassemia. Am J Ophthalmol. 1994;117:589–92.CrossRefPubMed
13.
Fanny A, Coulibaly F, Gbe K, Meite M, Adjorlolo C, Konan-Toure ML, Berete R, Boni S, Ouattara A, Diallo M. Sickle cell beta-thalassemia leading to serious ischemic retinopathy: a study of 18 patients in Abidjan. J Fr Ophtalmol. 2005;28:391–5.CrossRefPubMed
14.
Bonkovsky HL, Davidoff A, Stark DD. Hepatic iron concentration and total body iron stores in thalassemia major. N Engl J Med 2000; 343:1656. author reply 1657.
15.
Hadziahmetovic M, Pajic M, Grieco S, Song Y, Song D, Li Y, Cwanger A, Iacovelli J, Chu S, Ying G, Connelly J, Spino M, Dunaief J. The oral Iron Chelator Deferiprone protects against retinal degeneration induced through diverse mechanisms. Transl Vis Sci Technol. 2012;1:7.CrossRefPubMedPubMedCentral
16.
Song D, Song Y, Hadziahmetovic M, Zhong Y, Dunaief JL. Systemic administration of the iron chelator deferiprone protects against light-induced photoreceptor degeneration in the mouse retina. Free Radic Biol Med. 2012;53:64–71.CrossRefPubMedPubMedCentral
17.
Song D, Zhao L, Li Y, Hadziahmetovic M, Song Y, Connelly J, Spino M, Dunaief JL. The oral iron chelator deferiprone protects against systemic iron overload-induced retinal degeneration in hepcidin knockout mice. Invest Ophthalmol Vis Sci. 2014;55:4525–32.CrossRefPubMedPubMedCentral
18.
Pan Y, Keane PA, Sadun AA, Fawzi AA. Optical coherence tomography findings in deferasirox-related maculopathy. Retin Cases Brief Rep. 2010;4:229–32.CrossRefPubMed
19.
Walia HS, Yan J. Reversible retinopathy associated with oral deferasirox therapy. BMJ Case Rep. 2013; 2013. pii: bcr2013009205.
20.
Davies SC, Marcus RE, Hungerford JL, Miller MH, Arden GB, Huehns ER. Ocular toxicity of high-dose intravenous desferrioxamine. Lancet. 1983;2:181–4.CrossRefPubMed
21.
Cohen A, Martin M, Mizanin J, et al. Vision and hearing during deferoxamine therapy. J Pediatr. 1990;117:326–30.CrossRefPubMed
22.
Baath JS, Lam WC, Kirby M, Chun A. Deferoxamine-related ocular toxicity: incidence and outcome in a pediatric population. Retina. 2008;28:894–9.CrossRefPubMed
23.
Viola F, Barteselli G, Dell'arti L, Vezzola D, Villani E, Mapelli C, Zanaboni L, Cappellini MD, Ratiglia R. Abnormal fundus autofluorescence results of patients in long-term treatment with deferoxamine. Ophthalmology. 2012;119:1693–700.CrossRefPubMed
24.
Viola F, Barteselli G, DellʼArti L, Vezzola D, Mapelli C, Villani E, Ratiglia R. Multimodal imaging in deferoxamine retinopathy. Retina. 2014;34:1428–38.CrossRefPubMed
25.
Gonzales CR, Lin AP, Engstrom RF, Kreiger AE. Bilateral vitelliform maculopathy and deferoxamine toxicity. Retina. 2004;24:464–7.CrossRefPubMed
26.
27.
Rahi AH, Hungerford JL, Ahmed AI. Ocular toxicity of desferrioxamine: light microscopic histochemical and ultrastructural findings. Br J Ophthalmol. 1986;70:373–81.CrossRefPubMedPubMedCentral
28.
Reddy S, Iturralde D, Meyerle C, Gross NE, Yannuzzi LA. Fundus autofluorescence in retinopathy caused by deferoxamine toxicity. Retin Cases Brief Rep. 2007;1:120–2.CrossRefPubMed
29.
Pall H, Blake DR, Winyard P, Lunec J, Williams A, Good PA, Kritzinger EE, Cornish A, Hider RC. Ocular toxicity of desferrioxamine-an example of copper promoted auto-oxidative damage? Br J Ophthalmol. 1989;73:42–7.CrossRefPubMedPubMedCentral
30.
Chaston TB, Richardson DR. Iron chelators for the treatment of iron overload disease: relationship between structure, redox activity, and toxicity. Am J Hematol. 2003;73:200–10.CrossRefPubMed
31.
Klettner A, Koinzer S, Waetzig V, Herdegen T, Roider J. Deferoxamine mesylate is toxic for retinal pigment epithelium cells in vitro, and its toxicity is mediated by p38. Cutan Ocul Toxicol. 2010;29:122–9.CrossRefPubMed
32.
Dettoraki M, Kattamis A, Ladas I, Maragkos K, Koutsandrea C, Chatzistefanou K, Laios K, Brouzas D, Moschos MM. Electrophysiological assessment for early detection of retinal dysfunction in β-thalassemia major patients. Graefes Arch Clin Exp Ophthalmol. 2017;255:1349–58.CrossRefPubMed
33.
34.
Jiang C, Hansen RM, Gee BE, Kurth SS, Fulton AB. Rod and rod mediated function in patients with beta-thalassemia major. Doc Ophthalmol. 1999;96:333–45.CrossRef
35.
Bui KM, Sadda SR, Salehi-Had H. Pseudovitelliform maculopathy associated with deferoxamine toxicity: multimodal imaging and electrophysiology of a rare entity. Digit J Ophthalmol. 2017;23:11–5.CrossRefPubMedPubMedCentral
36.
Roulez F. Retinal pigment epithelium-desferal. Bull Soc Belge Ophtalmol. 2007;304:59–66.
37.
Kertes PJ, Lee TK, Coupland SG. The utility of multifocal electroretinography in monitoring drug toxicity: deferoxamine retinopathy. Can J Ophthalmol. 2004;39:656–61.CrossRefPubMed
38.
Genead MA, Fishman GA. Efficacy of brinzolamide ophthalmic suspension 1% for treatment of a vitelliform macular lesion in a patient with desferrioxamine retinopathy. Ophthalmic Surg Lasers Imaging 2011;42:e114–e117.Online.
Metadata
Title
Pattern dystrophies in patients treated with deferoxamine: report of two cases and review of the literature
Authors
Constantine D. Georgakopoulos
Foteini Tsapardoni
Elli V. Kostopoulou
Olga E. Makri
Publication date
01-12-2018
Publisher
BioMed Central
Published in
BMC Ophthalmology / Issue 1/2018
Electronic ISSN: 1471-2415
DOI
https://doi.org/10.1186/s12886-018-0911-2

Other articles of this Issue 1/2018

BMC Ophthalmology 1/2018 Go to the issue

Research article

Real-world refractive outcomes of toric intraocular lens implantation in a United Kingdom National Health Service setting

Research article

UVB-mediated down-regulation of proteasome in cultured human primary pterygium fibroblasts

Research article

Effect of number and position of intraocular lens haptics on anterior capsule contraction: a randomized, prospective trial

Research article

Identification of H2O2 induced oxidative stress associated microRNAs in HLE-B3 cells and their clinical relevance to the progression of age-related nuclear cataract

Case report

Eye damage due to cosmetic ultrasound treatment: a case report

Research article

Prevalence of refractive errors in Tibetan adolescents