κ I light chain AL amyloidosis presenting with rapidly progressive renal and hepatic failure with unusual renal amyloid distribution

Yuji Oe, Izaya Nakaya, Mayumi Yahata, Okinora Murata, Hiroshi Yaegashi, Tsutomu Sakuma, Hiroshi Sato, Juris J. Liepnieks, Merrill D. Benson, Jun Soma

Research output: Contribution to journalArticlepeer-review

2 Scopus citations

Abstract

A 65-year-old man suffering from generalized edema and jaundice was admitted to our hospital. Laboratory findings revealed marked renal dysfunction with heavy proteinuria as well as liver dysfunction with severe obstructive jaundice. On renal biopsy, the diagnosis of AL amyloidosis associated with κ I light chain was made. Interestingly, amyloid deposits were restricted to the glomeruli. Although hemodialysis was initiated, the patient died due to further deterioration of hepatic function. On autopsy, severe intrahepatic cholestasis was observed, and there was marked deposition of AL amyloid in the liver. Literature reviews showed that rapidly progressive renal failure is common in AL amyloidosis patients who presented with acute hepatic failure due to severe intrahepatic cholestasis. However, the detailed renal pathology in this condition has not been documented. The present case is very interesting because rapidly progressive renal and hepatic failure was simultaneously observed, and renal amyloid deposition was restricted to the glomeruli.

Original languageEnglish (US)
Pages (from-to)66-70
Number of pages5
JournalClinical Nephrology
Volume77
Issue number1
DOIs
StatePublished - Jan 1 2012

Keywords

  • κ Light chain
  • Acute hepatic failure
  • Acute renal failure
  • AL amyloidosis
  • Obstructive jaundice

ASJC Scopus subject areas

  • Nephrology

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