Secondary haemochromatosis

Case contributed by Dr Abdallah Khateeb

Presentation

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

Patient Data

Age: 21 years old
Gender: Male
CT

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 haemochromatosis, with the cells of the reticloendothelial system (RES) being the main reservoir.

In the case of secondary haemochromatosis, 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 haematopoiesis.

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

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Case information

rID: 44914
Case created: 8th May 2016
Last edited: 6th Mar 2017
Inclusion in quiz mode: Included

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