title: Vasotropic light-chain amyloidosis and ischaemic cholangiopathy.
abstract: 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.
authors.affiliation: Department of Gastroenterology, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, UK.
authors.forename: Emma L
authors.initials: EL
authors.lastname: Johnston
authors.affiliation: Department of Gastroenterology, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, UK.
authors.forename: Mark
authors.initials: M
authors.lastname: Wilkinson
authors.affiliation: Institute of Liver Studies, King's College Hospital, London, UK.
authors.forename: A S
authors.initials: AS
authors.lastname: Knisely
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000175:diagnosis* / Q000378:metabolism; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001652:Bile Ducts / Q000378:metabolism / Q000473:pathology; D001706:Biopsy; D001853:Bone Marrow / Q000378:metabolism / Q000473:pathology; D003520:Cyclophosphamide / Q000627:therapeutic use; D003907:Dexamethasone / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000097:blood / Q000378:metabolism*; D008099:Liver / Q000378:metabolism / Q000473:pathology; D008111:Liver Function Tests; D009101:Multiple Myeloma / Q000175:diagnosis* / Q000378:metabolism; D000683:Serum Amyloid P-Component / Q000378:metabolism*; D013154:Spleen / Q000378:metabolism / Q000473:pathology
chemical_list: D000970:Antineoplastic Agents; D007145:Immunoglobulin kappa-Chains; D000683:Serum Amyloid P-Component; D003907:Dexamethasone; D003520:Cyclophosphamide