Extramedullary hematopoiesis in β-thalassemia major patient: a case report and review of the literature

Extramedullary hematopoiesis (EMH), as a compensatory phenomenon, refers to the blood cell formation outside of the bone marrow that occurs once the cells in the circulatory system fail to meet individuals' needs. EMH is rare in moderate to severe beta thalassemia because most symptomatic patients are effectively managed with transfusion. However, patients that fail to receive transfusions like beta-thalassemia intermedia (beta-TI) as indicated are at increased risk for developing EMH. This paper describes the case of a 15-year-old female adolescent with beta-thalassemia major (beta-TM), suffering from a rare form of EMH affecting the sinus cavities, characterized by headache, sinusitis, and nasal obstruction, as confirmed by physical-pathological examinations and computerized tomography (CT) scan findings. The EMH in this patient could be significantly attributed to the lack of regular blood transfusions in recent years. It was concluded that beta-TM along with the occurrence of EMH in the sinus cavities had led to a complex case, carrying a heavy burden of the disease for the patient.