Hemochromatosis (pancreatic manifestations)

Changed by Rohit Sharma, 23 Jun 2018

Updates to Article Attributes

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Pancreatic manifestations of haemochromatosis typically occur with primary haemochromatosis, as the organ is usually spared in the secondary form of the disease.

For a general discussion, and for links to other system specific manifestations, please refer to the article on haemochromatosis.

Clinical presentation

Patients present with diabetes mellitus 4, but tend to also exhibit other manifestations of haemochromatosis, such as hepatic or cardiac dysfunction.

Pathology

Iron deposition can occur in the pancreas with primary hemochromatosis. Pancreatic involvement is uncommon in patients without liver cirrhosis. Most cirrhotic patients with primary hemochromatosis have pancreatic iron deposition and may have type 1 diabetes mellitus

Radiographic features

MRI

Iron causes magnetic susceptibility artefact, which leads to spin dephasing (T2*-related signal loss). This dephasing results in decreased signal intensity on MRI images. Like the liver, the pancreas will be markedly hypointense on T2 weighted- and T1-weighted T2* gradient echo sequences.1,3.

Dual sequences MR imaging (gradient in and out phase) demonstrates decreased signal intensity in the iron-loaded tissues on the in-phase images compared with the out-phase images (opposite of what you would see with microscopic fat deposit as in hepatic steatosis). This occurs because the echo time of the in-phase sequence is usually higher than that of the out-phase sequence; therefore, the in-phase pulse sequence is more sensitive to iron deposits because of the increased T2* effect 2.

  • -<p><strong>Pancreatic manifestations of haemochromatosis</strong> typically occur with primary <a href="/articles/haemochromatosis">haemochromatosis</a>, as the organ is usually spared in the secondary form of the disease. </p><h4>Pathology</h4><p>Iron deposition can occur in the <a href="/articles/pancreas">pancreas</a> with primary hemochromatosis. Pancreatic involvement is uncommon in patients without <a href="/articles/cirrhosis">liver cirrhosis</a>. Most cirrhotic patients with primary hemochromatosis have pancreatic iron deposition and may have <a href="/articles/diabetes-mellitus">type 1 diabetes mellitus</a>. </p><h4>Radiographic features</h4><h5>MRI</h5><p>Iron causes magnetic susceptibility artefact, which leads to spin dephasing (<a href="/articles/t2-relaxation-1">T2*-related signal loss</a>). This dephasing results in decreased signal intensity on MRI images. Like the liver, the pancreas will be markedly hypointense on T2 weighted- and T1-weighted T2* gradient echo sequences.<sup>1,3 </sup></p><p>Dual sequences MR imaging (<a href="/articles/in-phase-and-out-of-phase-sequences-1">gradient in and out phase</a>) demonstrates decreased signal intensity in the iron-loaded tissues on the in-phase images compared with the out-phase images (opposite of what you would see with microscopic fat deposit as in <a href="/articles/hepatic-steatosis">hepatic steatosis</a>). This occurs because the echo time of the in-phase sequence is usually higher than that of the out-phase sequence; therefore, the in-phase pulse sequence is more sensitive to iron deposits because of the increased T2* effect 2.</p>
  • +<p><strong>Pancreatic manifestations of haemochromatosis</strong> typically occur with primary <a href="/articles/haemochromatosis">haemochromatosis</a>, as the organ is usually spared in the secondary form of the disease.</p><p>For a general discussion, and for links to other system specific manifestations, please refer to the article on <a href="/articles/haemochromatosis">haemochromatosis</a>.</p><h4>Clinical presentation</h4><p>Patients present with diabetes mellitus <sup>4</sup>, but tend to also exhibit other manifestations of <a href="/articles/haemochromatosis">haemochromatosis</a>, such as hepatic or cardiac dysfunction.</p><h4>Pathology</h4><p>Iron deposition can occur in the <a href="/articles/pancreas">pancreas</a> with primary hemochromatosis. Pancreatic involvement is uncommon in patients without <a href="/articles/cirrhosis">liver cirrhosis</a>. Most cirrhotic patients with primary hemochromatosis have pancreatic iron deposition and may have <a href="/articles/diabetes-mellitus">type 1 diabetes mellitus</a>. </p><h4>Radiographic features</h4><h5>MRI</h5><p>Iron causes magnetic susceptibility artefact, which leads to spin dephasing (<a href="/articles/t2-relaxation-1">T2*-related signal loss</a>). This dephasing results in decreased signal intensity on MRI images. Like the liver, the pancreas will be markedly hypointense on T2 weighted- and T1-weighted T2* gradient echo sequences <sup>1,3</sup>.</p><p>Dual sequences MR imaging (<a href="/articles/in-phase-and-out-of-phase-sequences-1">gradient in and out phase</a>) demonstrates decreased signal intensity in the iron-loaded tissues on the in-phase images compared with the out-phase images (opposite of what you would see with microscopic fat deposit as in <a href="/articles/hepatic-steatosis">hepatic steatosis</a>). This occurs because the echo time of the in-phase sequence is usually higher than that of the out-phase sequence; therefore, the in-phase pulse sequence is more sensitive to iron deposits because of the increased T2* effect <sup>2</sup>.</p>

References changed:

  • 4. Utzschneider KM, Kowdley KV. Hereditary hemochromatosis and diabetes mellitus: implications for clinical practice. (2010) Nature reviews. Endocrinology. 6 (1): 26-33. <a href="https://doi.org/10.1038/nrendo.2009.241">doi:10.1038/nrendo.2009.241</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/20010968">Pubmed</a> <span class="ref_v4"></span>

Systems changed:

  • Haematology

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