Barbaros KARAGÜN, Yurdanur KILINÇ, İlgen ŞAŞMAZ, Ali ANTMEN

Talasemi Majörlü Bir Hastada Deferipron Kullanımına Bağlı Gelişen Katarakt

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.

Anahtar Kelimeler:

Talasemi; katarakt; deferibron: çocuk

Cataract Formation due to use of Deferiprone in a Patient with Thalassemia Major

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.

Keywords:

Thalassemia; cataract; deferibron; child,

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