İlk prezentasyonu otalji olan beta talasemi: olgu sunumu

Beta talasemi gen mutasyonlarının sebep olduğu hematolojik bir hastalıktır ve Türkiye’yi de içeren tüm Akdeniz ülkelerinde önemli bir halk sağlığı problemi teşkil etmektedir. Talasemi intermedia talasemi major ve talasemi minor arasındaki bir ara formdur. Temporal kemik tutulumu talasemi intermediada oldukça nadirdir. Literatürde otalji ile birlikte olan ve talasemi minor tanısı alan vaka bulunmamaktadır. Bizim hastamız 10 aydır sol kulak ağrısı ve işitme kaybı çekmektedir. Bu vakada, yapılan tetkikler sonucunda talasemi intermedia tanısı alan ve manyetik rezonans görüntülemede temporal kemik tutulumu olan 44 yaşında bir erkek hasta sunulmaktadır.

Otalgia as the first presenting complaint in a patient with beta thalassemia: Case Report

Beta thalassemia is a hematological disease caused by gene mutations and represents an important public health problem in all Mediterranean countries, including Turkey. Thalassemia intermedia is an intermediate form between thalassemia major and thalassemia minor. Temporal bone involvement is quite rare in thalassemia intermedia. There are no cases in the literature who presented with otalgia and was subsequently diagnosed with thalassemia intermedia. Our patient had suffered from left ear pain and hearing loss for 10 months. In this case report, a 44-year-old male patient is presented who was diagnosed with thalassemia intermedia based on the examinations performed and had temporal bone involvement on magnetic resonance imaging.

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  • Galanello R, Origa R. Beta-thalassemia. Orphanet J Rare Dis. 2010; 21: 5-11.
  • Karakas Z. Beta Talasemi Intermedia. Turkiye Klinikleri J Pediatr Sci. 2007; 3: 34-41.
  • Aessopos A, Kati M, Meletis J Thalassemia intermedia today: should patients regularly receive transfusions? Transfusion. 2007; 47: 792-800.
  • Taher A, Isma'eel H, Cappellini MD. Thalassemia intermedia: revisited. Blood Cells Mol Dis. 2006; 37:12-20.
  • Malik M, Pillai LS, Gogia N, Puri T, Mahapatra M, Sharma DN, Kumar R. Paraplegia due to extramedullary hematopoiesis in thalassemia treated successfully with radiation therapy. Haematologica. 2007; 92: 28-30.
  • Castelli R, Graziadei G, Karimi M, Cappellini MD Intrathoracic masses due to extramedullary hematopoiesis. Am J Med Sci. 2004; 328: 299-303.
  • Sheikha A, Kameswaran M, Okafor BC, al-Saigh AA. Otological manifestations of thalassaemia intermedia: evidence of temporal bone involvement and report of a unique cholesteatoma-like lesion. J Laryngol Otol. 1992; 106: 316-321.