2025 Volume 46 Issue 1 Pages 42-47
Thalassemia is a congenital disorder that causes hemolytic anemia due to impaired hemoglobin synthesis. Thalassemia major is very rare and hematopoiesis is enhanced by prolonged hemolysis, which may be complicated by myeloid hyperplasia. The patient is a 13-year-old boy. When he was 4 years old, he was diagnosed with beta thalassemia and had been under observation since then. When he was 13 years old, bilateral buccal swelling began to appear and he was referred to our hospital. He had nasal stenosis but no respiratory impairment. In addition, he presented with bilateral otitis media with effusion and conductive hearing loss. CT scan revealed myeloid hyperplasia of the maxilla and perimeter of the auditory tube to mastoid air cells, but no obvious deformity of the auditory ossicles. We performed insertion of transtympanic ventilation tube on him, and conductive hearing loss was improved. However, hearing loss in the low frequency range remained. The curative treatment for thalassemia was a bone marrow transplant, which was performed when he was 16 years old. Even after bone marrow transplant, there is a possibility of respiratory impairment and progressive hearing loss due to increased facial myeloid hyperplasia. Therefore, long-term follow-up is necessary.
