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CASE REPORT
Vasotropic light-chain amyloidosis and ischaemic cholangiopathy
  1. Emma L Johnston1,
  2. Mark Wilkinson1,
  3. A S Knisely2
  1. 1Department of Gastroenterology, Guy's and St Thomas’ Hospitals NHS Foundation Trust, London, UK
  2. 2Institute of Liver Studies, King's College Hospital, London, UK
  1. Correspondence to Dr Emma L Johnston, emma.johnston{at}gstt.nhs.uk

Summary

A 75-year-old woman was incidentally found to have deranged liver function tests (LFTs). She was well, apart from 2 years of dyspnoea. Investigations had revealed atrial fibrillation and a right pleural effusion, without identified aetiology. On examination, the only finding was a palpable liver edge. Initial blood and ultrasound screening suggested no cause. The patient underwent liver biopsy. Microscopy showed κ-immunoglobulin light chains deposited exclusively in portal tracts, within blood vessel and bile duct walls. This pattern, although unusual, raised the possibility of κ-light chain disease. Serum electrophoresis was normal, as were serum immunoglobulin values. Serum concentrations of κ-light chains were elevated and microscopy of aspirated bone marrow found light-chain deposits with 10% plasmacytosis. Serum amyloid P (SAP) scintigraphy demonstrated splenic uptake. Myeloma, κ-light chain, with light-chain amyloidosis was diagnosed. The patient has responded well to cyclophosphamide, bortazomib and dexamethasone chemotherapy, and her LFTs are now nearly normal.

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