Liver transplantation for liver rupture due to light chain deposition disease: A case report

Anca G. Croitoru, Prodromos Hytiroglou, Myron E. Schwartz, Romil Saxena

Research output: Contribution to journalArticle

12 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)298-303
Number of pages6
JournalSeminars in Liver Disease
Volume26
Issue number3
DOIs
StatePublished - Aug 2006

Fingerprint

Liver Transplantation
Rupture
Light
Liver
Amyloidosis
Kidney
Congo Red
Paraproteinemias
Hepatomegaly
Amyloid Plaques
Atrophy
Liver Diseases
Coloring Agents
Blood Platelets

Keywords

  • Amyloid
  • Hepatomegaly
  • Light chains
  • Rupture
  • Transplantation

ASJC Scopus subject areas

  • Hepatology

Cite this

Liver transplantation for liver rupture due to light chain deposition disease : A case report. / Croitoru, Anca G.; Hytiroglou, Prodromos; Schwartz, Myron E.; Saxena, Romil.

In: Seminars in Liver Disease, Vol. 26, No. 3, 08.2006, p. 298-303.

Research output: Contribution to journalArticle

Croitoru, Anca G. ; Hytiroglou, Prodromos ; Schwartz, Myron E. ; Saxena, Romil. / Liver transplantation for liver rupture due to light chain deposition disease : A case report. In: Seminars in Liver Disease. 2006 ; Vol. 26, No. 3. pp. 298-303.
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