Journal of Postgraduate Medicine
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Year : 2011  |  Volume : 57  |  Issue : 1  |  Page : 61-62  

Amyloidosis of exocrine pancreas

P Vaideeswar1, R Chetty2,  
1 Department of Pathology (Cardiovascular & Thoracic Division), Seth GS Medical College, Mumbai, India
2 Institute of Cancer Sciences, College of Medicine, Veterinary & Life Sciences, University of Glasgow, Scotland

Correspondence Address:
P Vaideeswar
Department of Pathology (Cardiovascular & Thoracic Division), Seth GS Medical College, Mumbai

How to cite this article:
Vaideeswar P, Chetty R. Amyloidosis of exocrine pancreas.J Postgrad Med 2011;57:61-62

How to cite this URL:
Vaideeswar P, Chetty R. Amyloidosis of exocrine pancreas. J Postgrad Med [serial online] 2011 [cited 2022 Nov 26 ];57:61-62
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Full Text

Amyloidosis represents a disease characterized by deposition of a variety of proteins as an insoluble, extracellular, hyaline material termed as amyloid. Irrespective of the type of the protein forming it, amyloid exhibits two important characteristics - positive staining with Congo red with apple-green birefringence under polarized light, and its arrangement as nonbranching beta-pleated crossed sheets. [1] In a systemic involvement, amyloidosis commonly affects the kidneys, spleen, and heart. [2] In this report, we present a case of extensive amyloid deposition of the exocrine pancreas, an organ which is very rarely affected.

A 40-year-old woman, with a history of chronic alcoholism, was hospitalized for multiple episodes of hemoptysis. Twenty days prior to her current admission, she had been diagnosed as a case of ischemic cardiomyopathy (mild global hypokinesis and left ventricular ejection fraction of 45%) with degenerative sinus node dysfunction and left bundle branch block. An ultrasonography had also suggested liver parenchymal disease, ascites, bilateral mild pleural effusions, and medical renal disease. She died within 40 minutes. No investigations had been performed.

A complete autopsy was performed. The heart (320 g) had features of dilated cardiomyopathy with moderate cardiomegaly, globular shape, and moderate biventricular dilatation. The lungs showed multifocal intrapulmonary hemorrhages. Liver was fatty; there was no cirrhosis or splenomegaly. The pancreas was small in size [Figure 1]a and exceedingly firm in consistency. There was retention of the lobular architecture, and the lobules were separated by translucent, glassy, gray-white connective tissue; similar appearance was noted in the peripancreatic fat and around lymph nodes and splenic artery [Figure 1]b. On histology, the perilobular connective tissue had a deposition of pink, acellular, homogeneous material [Figure 1]c. This was also present within the lobules, separating the pancreatic acini, surrounding ductules/ducts, but spared the islets [Figure 2]. The material retained its "Congophilia" despite permanganate pretreatment (ruling out secondary amyloidosis) and showed apple-green birefringence under polarized light [Figure 2]. Pericellular deposits were found in the myocardium, along with pulmonary and systemic arterial involvement. Glomeruli were spared; bone marrow was normal. {Figure 1}{Figure 2}

We have reported a case with extensive deposition of amyloid in the exocrine part of the pancreas, that is, "extrainsular" involvement. The term "extrainsular" has been used because in most instances, the amyloid is deposited in the islets or "insular." The latter forms an example of localized amyloidosis, seen in type 2 diabetes mellitus and insuliomas, where the amyloid protein is islet amyloid polypeptide or amylin. [3],[4] Infrequently, islets can be the site of primary amyloidosis. [5] Exocrine pancreatic involvement in systemic amyloidosis is said to be uncommon, but is likely that this aspect is not well documented at autopsy; besides, there is usually no alteration in exocrine or endocrine function. [6] The pancreas at autopsy was small in size, in contrast to the finding of enlarged pancreas in few reported cases. [6] The index case had no other pathology which could explain systemic amyloid deposition. Although there was no apparent immune dyscrasia (normal bone-marrow histology), we have presumed that this is a primary process in view of persistence of Congo-red staining even after permanganate treatment, young age of the patient, prominent involvement of the heart, and diffuse pulmonary and vascular affection.


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