Extramedullary Hematopoiesis of the Maxilla in Beta-Thalassemia

Abstract

Objective: 1) Present a rare case of diffuse craniofacial compensatory marrow expansion in a patient with β-thalassemia resulting in near-complete obliteration of bilateral maxillary sinuses and partial nasal obstruction. 2) Learn about compensatory extramedullary hematopoeisis in patients with beta-thalassemia and its effects on the maxillae and paranasal sinuses.

Method: A 41-year-old female with β-thalassemia presented to the otolaryngology clinic for recurrent epistaxis. The patient was interviewed and examined, and the medical record and radiographic images were reviewed. The patient was observed for 6 months. Literature review of similar cases was performed.

Results: Nasal endoscopy revealed an apparent mass within the right nasal cavity. CT of the sinuses showed diffuse marrow expansion resulting in complete obliteration of the right maxillary sinus, a small residual left maxillary sinus, partial nasal obstruction, and septal deviation. The radiologist described the appearance as consistent with extramedullary hematopoeisis. Despite the extensive bone remodeling, the patient’s remaining sinuses appeared clear, and she was minimally symptomatic. The appearance was stable as compared to imaging obtained 5 years earlier during an unrelated diagnostic workup. We elected to conservatively manage her epistaxis and follow her for worsening symptoms or clinical exam findings.

Conclusion: β-thalassemia causes characteristic changes of the maxilla that should be recognized on CT. The expansion of hematopoietic tissue may contribute to nasal or sinus symptoms. If asymptomatic, these abnormalities can safely be observed. The cornerstone of treatment for β-thalassemia patients is frequent blood transfusions to suppress additional hematopoiesis.