Secondary hemochromatosis

Case contributed by Abdallah Al Khateeb


Known case of thalassemia with recurrent blood transfusions. Old splenectomy due to trauma.

Patient Data

Age: 20 years old
Gender: Male

The liver is enlarged and of abnormal diffuse high attenuation (average HU = 90). The vascularity and biliary tree are more obvious than usual, due to their relative lower attenuation.

Generalized osteopenia is noted on the bone window images.

An abnormal soft tissue seen at the presacral region.

The pancreas is unremarkable.

Few small dense gallbladder stones.

Right side double J stent, due to recent extracorporeal shock wave lithotripsy of previous right pelvicalyceal stones.

Case Discussion

Following repeated blood transfusions, this patient developed secondary iron overload or secondary hemochromatosis, with the cells of the reticuloendothelial system (RES) being the main reservoir.

In the case of secondary hemochromatosis, iron will be building up in both RES and non-RES tissues, which sometimes helps differentiate both forms radiologically.

The bony changes in this patient are thought to be the effect of chronic severe anemia, and the presacral soft tissue is best explained by extramedullary hematopoiesis.

This case would be a classic for a fellowship viva or long case, such as in the FRCR 2b exam.

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