Eextramedullary haematopoesis - adrenal

Case contributed by Professor Ali Hekmatnia

Presentation

Known case of beta-thalassemia major with incidental finding of right adrenal mass in follow up ultrasound

Patient Data

Age: 40 years
Gender: Female

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 hemosiderin rim.

Case Discussion

Our patient has beta thalassemia 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 tumor of adrenal glands and is asymptomatic. Therefore, diagnosis is incidental and exists from birth in contrast with extramedullary hematopoiesis which develops over time.

Due to these reasons, our diagnosis is extramedullary hematopoiesis in the adrenal glands. In addition, myelolipoma is included in differential diagnoses and always needs to be considered.

Extramedullary hematopoiesis (EMH) could be presented with several hematological disorders such as major beta-thalassemia. Myelolipomas are usually composed of fat and hematopoietic cells include myeloid, erythroid, and megakaryocytes. Myelolipoma originates from reticuloendothelial cells of capillaries and is the main differential diagnosis of extramedullary hematopoiesis.

PlayAdd to Share

Case information

rID: 66711
Published: 23rd Mar 2019
Last edited: 24th Mar 2019
Inclusion in quiz mode: Included
Institution: Isfahan University of Medical Sciences

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.