Abstract
Thalassemias are a heterogeneous group of autosomal recessive diseases characterized by hypochromic microcytic anemia and occur as a result of defective synthesis of one or more hemoglobin chains. In patients, life-threatening clinical manifestations may occur because of severe iron overload due to frequent blood transfusions. Ocular changes in patients with thalassemia are to be encountered depending on the disease itself or chelator used in the treatment, but not very often. These changes are usually cataracts, optic neuropathy, retinal pigment epithelium (RPE) degeneration, RPE mottling, retinal venous tortuosity, vitreoretinal hemorrhages and obliteration of the iris pattern. Desferrioxamine that is used as the first iron-binding chelating has well-known complications such as optic neuropathy and retinal toxicity. However, Deferiprone that used more common recently has replaced the Desferrioxamine but, there is very little information in the literature about the ocular toxicity of deferiprone. In this case report, we have reported a patient with deferiprone-induced cataract formation in order to draw attention to a little-known complication of the drug.
References
- Kutlu M, Çekmiş H, Başak M, Osman N, Açıkgöz Ö. Talasemiler.
- Bakırköy Tıp Dergisi. 2006;2:33-40.
- Yeşilipek A. Talasemi Tedavisindeki Gelişlmeler.
- Güncel Pediatri Dergisi. 2005;3:1
- Mehdizadeh M, Nowroozzadeh MH. Posterior subcapsular opacity in two patients with thalassaemia major following deferiprone consumption. Clin Exp Optom. 2009; 92:392-4.
- 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.
- Gaba A, Souza PD, Chandra J, Narayan S, Sen S. Ocular changes in beta thalassemia. Ann Ophthalmol. 1998;30:357–60.
- Gartaganis S, Ismiridis K, Papageorgiou O. Ocular abnormalities in patients with beta thalassemia. Am J Ophthalmol. 1989;108:699–703.
- 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.
- Aessopos A, Floudas CS, Kati M, Tsironi M, Giakoumi X, Livir-Rallatos C, Farmakis D. Loss of vision associated with angioid streaks in beta- thalassemia intermedia. Int J Hematol. 2008;87:35–8.
- Olivieri NF, Buncic JR, Chew E, Gallant T, Harrison RV, Keenan N, Logan W et al. Visual and auditory neurotoxicity in patients receiving subcutaneous deferoxamine infusions. N Engl J Med. 1986;314:869–73.
- Cohen AR, Galanello R, Pennell DJ, Cunningham MJ, Vichinsky E. Thalassemia. Hematology Am Soc
- Hematol Educ Program. 2004;14–34.
Abstract
Talasemiler; otozomal resesif kalıtım gösteren, hemoglobin zincirlerinden birinin veya bir kaçının hasarlı sentezi sonucu ortaya çıkan hipokrom mikroster anemi ile karakterize heterojen bir grup hastalıktır. Hastaların sık transfüzyona maruz kalması sonucunda aşırı demir birikimine bağlı yaşamı tehdit eden önemli klinik bulgular ortaya çıkar. Talasemili hastalarda hastalığın kendisine ya da kullanılan şelatör tedavisine bağlı olarak oküler değişikliklere çok sık olmasa da rastlanmaktadır. Bu değişiklikler genellikle katarakt, optik nöropati, retinal pigment epitelinde (RPE) dejenerasyon, RPE mottling, retinal venous tortuosity, vitreoretinal hemorrhages and obliteration of iris patern şeklindedir. İlk defa demir bağlayıcı şelatör olarak kullanılmaya başlanan deferoxamin optik nöropati ve retinal toksisite gibi iyi bilinen komplikasyonlara sahiptir. Ancak deferoxaminin yerini alan ve yakın dönemde sık kullanılan deferipronun oküler toksisitesi ile ilgili literatürde daha önce bildirilmiş az sayıda bilgi vardır. Bu olgu deferipron kullanımına bağlı gelişen katarakt oluşumuna dikkat çekmek amacıyla bildirilmiştir.
References
- Kutlu M, Çekmiş H, Başak M, Osman N, Açıkgöz Ö. Talasemiler.
- Bakırköy Tıp Dergisi. 2006;2:33-40.
- Yeşilipek A. Talasemi Tedavisindeki Gelişlmeler.
- Güncel Pediatri Dergisi. 2005;3:1
- Mehdizadeh M, Nowroozzadeh MH. Posterior subcapsular opacity in two patients with thalassaemia major following deferiprone consumption. Clin Exp Optom. 2009; 92:392-4.
- 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.
- Gaba A, Souza PD, Chandra J, Narayan S, Sen S. Ocular changes in beta thalassemia. Ann Ophthalmol. 1998;30:357–60.
- Gartaganis S, Ismiridis K, Papageorgiou O. Ocular abnormalities in patients with beta thalassemia. Am J Ophthalmol. 1989;108:699–703.
- 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.
- Aessopos A, Floudas CS, Kati M, Tsironi M, Giakoumi X, Livir-Rallatos C, Farmakis D. Loss of vision associated with angioid streaks in beta- thalassemia intermedia. Int J Hematol. 2008;87:35–8.
- Olivieri NF, Buncic JR, Chew E, Gallant T, Harrison RV, Keenan N, Logan W et al. Visual and auditory neurotoxicity in patients receiving subcutaneous deferoxamine infusions. N Engl J Med. 1986;314:869–73.
- Cohen AR, Galanello R, Pennell DJ, Cunningham MJ, Vichinsky E. Thalassemia. Hematology Am Soc
- Hematol Educ Program. 2004;14–34.
Details
| Primary Language | English |
|---|---|
| Journal Section | Case Report |
| Authors | |
| Publication Date | October 9, 2015 |
| Published in Issue | Year 2015 Volume: 40 Number: Supplement 1 |
