Presentation
Known thalassemia patient on regular blood transfusion. Complaining of reduced growth and sex hormone levels.
Patient Data
The pituitary gland shows hypointense on T1 and T2 WS with no intravenous contrast enhancement, findings compatible with hemosiderin deposits.
Diffuse calvarial thickening eliciting low marrow signal.
Case Discussion
Central nervous system manifestations of hemochromatosis are uncommon and can occur in either primary or secondary hemochromatosis.
In hemochromatosis, iron deposition in the brain is uncommon because the blood-brain barrier protects the brain from systemic iron overload, which means that siderosis generally occurs in regions without a blood-brain barrier, such as the choroid plexus and circumventricular organs 7-9. Additionally, iron deposition also commonly occurs in the anterior pituitary gland, predominantly in gonadotrophs, leading to hypogonadotropic hypogonadism, although reasons for anterior pituitary gland involvement are yet to be fully elucidated
On imaging, the pituitary gland can demonstrates a signal drop in the T2 WS of the neurohypophysis is pathognomonic. In severe cases, signal drop in T1 WS can also be observed, which might be accompanied by a decrease in pituitary volume. T2 * gradient echo (GRE) is the most sensitive sequence for the detection of iron deposition demonstrating a marked decrease in the signal intensity.
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