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Amyloidogenic Immunoglobulin Chain and Non-amyloidogenic Immunoglobulin Chain Diseases

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Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Abstract

B-cell non-Hodgkin’s lymphomas (NHL) can be associated with amyloidogenic immunoglobulin chain and non-amyloidogenic immunoglobulin chain disease. In both groups of disorders, the hepatobiliary tract can be involved in form of various patterns. Hepatic amyloidosis caused by NHL is termed amyloid light-chain or AL amyloidosis (primary amyloidosis) and develops in the setting of various B-cell neoplasms, most commonly with plasmacytoma. This form of amyloidosis induces an enlarged liver with increased transparency and elevated consistency. The predominant histological localization of amyloid is perisinusoidal and in portal tracts, associated with marked atrophy of hepatocyte plates. In a minority of cases of AL amyloidosis, tumor-like amyloid masses can develop (amyloidoma or amyloid tumor). This tumefactive hepatic amyloidosis is a very rare disorder. Amyloidosis can rarely involve intrahepatic and extrahepatic bile ducts. Among non-amyloidogenic immunoglobulin chain diseases, light-chain deposit disease can involve the liver. The liver is in fact the most frequent extrarenal localization of this disorder and is characterized by the deposition of granular or ribbonlike non-congophilic material along sinusoids.

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  1. University of Bern, Bern, Switzerland

    Arthur Zimmermann

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  1. Arthur Zimmermann
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Zimmermann, A. (2017). Amyloidogenic Immunoglobulin Chain and Non-amyloidogenic Immunoglobulin Chain Diseases. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_93

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  • DOI: https://doi.org/10.1007/978-3-319-26956-6_93

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