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Semin Liver Dis 2006; 26(3): 298-303
DOI: 10.1055/s-2006-947301
DIAGNOSTIC PROBLEMS IN HEPATOLOGY

Copyright © 2006 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Liver Transplantation for Liver Rupture Due to Light Chain Deposition Disease: A Case Report

Anca G. Croitoru1 , Prodromos Hytiroglou2 , Myron E. Schwartz3 , Romil Saxena4
  • 1Department of Pathology, University of Alabama at Birmingham
  • 2Department of Pathology, Aristotle University Medical School, Thessaloniki, Greece
  • 3Recanati Miller Transplantation Institute, Mount Sinai School of Medicine, New York, New York
  • 4Department of Pathology, Indiana University, Indianapolis, Indiana
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Publication History

Publication Date:
19 July 2006 (online)

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ABSTRACT

Light chain deposition disease (LCDD) is a rare pathologic condition distinct from amyloidosis. Amyloidosis is most often characterized by overproduction of lambda light chains, while kappa chains are overproduced in LCDD. In contrast to amyloid deposits, those of LCDD do not stain with Congo red and have a granular ultrastructure. LCDD primarily affects the kidney; clinically significant liver dysfunction is less common and less severe than renal disease. We describe a case of kappa chain deposition disease in a patient with plasma cell dyscrasia and platelet pool storage defect, which produced massive hepatomegaly and rupture of the liver leading to orthotopic liver transplantation. The liver weighed 6800 g and showed severe atrophy due to massive deposition of light chains. In this case, the deposits were composed of unbranched fibrils, which measured 12 to 20 nm in width, did not possess a hollow core, and were arranged randomly rather than in structured arrays.

KEYWORDS

Light chains - amyloid - transplantation - rupture - hepatomegaly

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Romil SaxenaM.B.B.S. F.R.C.Path. 

Department of Pathology and Laboratory Medicine, Indiana University

350 West 11th Street, #4014, Indianapolis, IN 46202

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