Duodenal biopsy: an unexpected confirmatory test for a patient with al-amyloidosis

S Pirasath,D D Dikowita,V Sujanitha,T Kumanan,G Selvaratnam

doi:10.4038/sljm.v28i1.101

Abstract

SSystemic AL- amyloidosis is a disorder of protein folding in which there is extra-cellular accumulation as β pleated fibrillar deposits of monoclonal immunoglobulin light chain fragments. AL- amyloidosis is a rare clinical entity. A presentation with clinical and histological evidence of gastrointestinal amyloidosis is even rare. Here we report primary AL- amyloidosis secondary to lamda (λ) light chain myeloma in a middle-aged woman who presented with nephrotic syndrome and motor peripheral neuropathy.

Highlights

Primary amyloid light chain (AL) amyloidosis is a rare variety of plasma cell dyscrasia, the diagnosis of which is often difficult to establish
There is extra cellular accumulation in β pleated fibrillar deposits of monoclonal immunoglobulin light chain fragments leading to organ failure
2 Here we report a case of primary AL amyloidosis due to lambda light chain myeloma in a middle-aged woman who presented with nephrotic syndrome and motor peripheral neuropathy

Summary

Introduction

Primary amyloid light chain (AL) amyloidosis is a rare variety of plasma cell dyscrasia, the diagnosis of which is often difficult to establish. There is extra cellular accumulation in β pleated fibrillar deposits of monoclonal immunoglobulin light chain fragments leading to organ failure. Pathogenesis of amyloidosis involves extracellular deposition of insoluble protein fibrils in tissues, leading to insufficiency of affected organs. Here we report a case of primary AL amyloidosis due to lambda light chain myeloma in a middle-aged woman who presented with nephrotic syndrome and motor peripheral neuropathy There is extra cellular accumulation in β pleated fibrillar deposits of monoclonal immunoglobulin light chain fragments leading to organ failure. 1 Pathogenesis of amyloidosis involves extracellular deposition of insoluble protein fibrils in tissues, leading to insufficiency of affected organs. 2 Here we report a case of primary AL amyloidosis due to lambda light chain myeloma in a middle-aged woman who presented with nephrotic syndrome and motor peripheral neuropathy

Case report

Discussion

Findings

The accurate diagnosis by biochemical analyses is essential for management of