Hemochromatosis with hyperdense liver

Case contributed by Ammar Ashraf
Diagnosis certain

Presentation

Abdominal pain. No fever, vomiting or change in bowel habits.

Patient Data

Age: 20 years
Gender: Male

Enlarged liver measuring 21.2 cm and showing homogenously increased parenchymal density (average density=95HU). Well distended gallbladder with multiple small radiopaque calculi; no signs of acute cholecystitis. The spleen is not visualized (past history of splenectomy).

The pancreas appears mildly atrophic. Multiple small soft tissue density mesenteric lymph nodes. Multiple small hyperdense left para-aortic lymph nodes (average density=64HU), particularly close to the left renal hilum.  Heterogeneous texture and coarse trabeculations in the scanned skeleton.

Enlarged liver with diffusely hypointense signal on T1 and T2 weighted images (lower than the paraspinal muscles and bones), in keeping with iron overload. No obvious focal hepatic lesion or biliary dilatation.

Gallbladder shows multiple tiny stones. Diffuse bone marrow signal loss in the visualized bony skeleton. Pancreas and kidneys also show signs of iron deposition. Hyperdense left para-aortic lymph nodes noted on CT scan also show diffusely low signal intensity on T1 and T2-weighted images. Status post splenectomy. 

Case Discussion

The patient is a known case of thalassemia major. There is a history of multiple repeated blood transfusions, leading to secondary hemochromatosis with complications (hypoparathyroidism, secondary adrenal insufficiency, and diabetes mellitus). Past history of splenectomy.

Increased hepatic attenuation on CT is likely related to multiple repeated blood transfusions, leading to secondary hemochromatosis. Increased density left para-aortic lymph nodes were also likely related to the same disease process.   

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