Intrathoracic and Presacral Extramedullary Haematopoiesis in a Thalassaemia Intermedia Patient

Abstract

Extramedullary Haematopoesis (EMH) is a normal physiologic phenomenon in inadequately transfused patients of chronic severe anaemia due to haemoglobinopathies or myelodysplasia. Although mostly asymptomatic, these haematopoetic cells rests when present along the paraspinal tissues may mimic intrathoracic and presacral masses producing pressure effects and confounding diagnoses. Intrathoracic EMH mostly develops in the posterior mediastinum. Presacral EMH is an extremely rare phenomenon with limited mention in medical literature. The precarious location leads to high risk of obtaining invasive biopsy and thus radiological and radionuclide scanning are the diagnostic modalities of choice. Intervention is only planned in symptomatic lesions. Therefore, the possibility of EMH must be kept in mind whenever diagnosing any mediastinal mass in a patient with a documented haemoglobinopathy. We present here such a case of intrathoracic and presacral EMH in a young lady with beta thalassaemia intermedia.

Keywords

• β thalassaemia intermedia
• mediastinal mass
• spinal cord compression