Presentation
Known case of beta-thalassaemia major with incidental finding of right adrenal mass in follow up ultrasound
Patient Data
Non-contrast enhanced CT-scan of the abdomen showing right adrenal fat containing heterogeneous mass with peripheral nodular soft tissue density, replacing it almost completely.
Hepatomegaly is also noted. History of splenectomy.
Magnetic resonance imaging (MRI) demonstrating a large right-sided predominantly fatty mass replacing the right adrenal gland which shows low signal intensity incomplete haemosiderin rim.
Case Discussion
Our patient has beta thalassaemia major and therefore, had regular ultrasound scans which were all normal and without any lesions in the adrenal glands and this lesion has been recently discovered.
However, adrenal myelolipoma is a tumour of adrenal glands and is asymptomatic. Therefore, diagnosis is incidental and exists from birth in contrast with extramedullary haematopoiesis which develops over time.
Due to these reasons, our diagnosis is extramedullary haematopoiesis in the adrenal glands. In addition, myelolipoma is included in differential diagnoses and always needs to be considered.
Extramedullary haematopoiesis (EMH) could be presented with several haematological disorders such as major beta-thalassaemia. Myelolipomas are usually composed of fat and haematopoietic cells include myeloid, erythroid, and megakaryocytes. Myelolipoma originates from reticuloendothelial cells of capillaries and is the main differential diagnosis of extramedullary haematopoiesis.