A case with primary amyloidosis of the liver and spleen: radiologic findings
Introduction
Primary amyloidosis is a rare disease characterized by the deposition of amyloid protein in many organs. Progressive organ involvement leads to organ malfunction and death usually resulting from renal and/or cardiac involvement. Although the liver and spleen are major sites of involvement, the clinical manifestations of hepatic and splenic involvement usually are mild and symptomatic disease including rupture or hepatic failure are rare [1], [2], [3], [4], [5], [6]. We report a case of primary amyloidosis of the liver and spleen with emphasis on radiologic findings including ultrasonographic and dynamic computed tomographic (CT) findings that have not been well described in the literature.
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Case report
A 73-year-old man presented with general fatigue and abdominal fullness. Physical examination revealed hepatomegaly. Laboratory examination revealed mild liver dysfunction and elevated CEA (7.9 ng/ml).
Hepatomegaly was noted on various imaging modalities. Ultrasonograms showed heterogeneous echogenicity in the liver (Fig. 1a). On precontrast phase scans of dynamic CT, the liver had diffuse decreased attenuation (Fig. 1b). Portal phase CT scans showed heterogeneous enhancement in the liver (Fig. 1
Discussion
Amyloidosis is characterized by the deposition of amyloid fibrillar proteins that are fairly resistant to normal proteolytic digestion. Progressive accumulation of amyloid compresses and replaces normal tissue, and it leads to organ dysfunction [1], [2], [3], [4]. Amyloidosis is usually seen in a systemic form, although 10–20% of cases can be localized [8]. Systemic amyloidosis is sub classified by a particular precursor protein into two major forms, primary amyloidosis (AL-amyloidosis) and
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