Immunoglobulin-derived light chain amyloidosis can occasionally be associated with localized disease. We present a patient with localized lymph node light chain amyloidosis without an underlying monoclonal protein or lymphoproliferative disorder and review the literature of lymph node amyloidosis discussing work-up and risk factors for systemic progression.

Primary systemic amyloidosis is a rare entity. We report a case of primary systemic primary amyloidosis, an elderly male presented with generalized weakness since 6 months. Clinical examination revealed typical waxy lesions in the periorbital area with macroglossia. Serum electrophoresis was normal, however, bone marrow examination showed increased plasma cell with a good number of both binucle...

CONTEXT Pancreatic involvement in systemic light chain (AL)-amyloidosis is exceedingly rare. Prior reports of endoscopic ultrasound (EUS) for the diagnosis of amyloidosis are also limited. CASE REPORT We report the first description of EUS-guided fine needle aspiration (FNA) for the diagnosis of primary AL-amyloidosis involving the pancreas. CONCLUSION EUS-FNA can be effectively utilized fo...

Systemic amyloidosis is a very rare complication of inflammatory bowel disease (IBD). The reported cases of secondary amyloidosis in children with IBD are much fewer than those reported in adults. Herein, a teenage boy with Crohn's disease is presented who developed nephrotic syndrome due to renal involvement secondary to amyloidosis, whereas the patient was under treatment with corticosteroid ...

BACKGROUND Amyloidosis includes a group of diseases characterized by the extracellular deposition of various fibrillary proteins that can autoaggregate in a highly abnormal fibrillary conformation. The amyloid precursor protein of systemic light-chain (AL) amyloidosis is comprised of monoclonal light chains that are due to plasma cell dyscrasia. The clinical presentation of patients with AL amy...

Amyloidosis is characterised by the extracellular deposition of abnormal protein fibrils. Recent advances in the study of amyloidosis have been based on the chemical analysis of these protein fibrils.1,2 This has also led to a more rational classification of the condition (Fig 1). Further discussion of localised forms of amyloidosis is outside the scope of this article, which will be confined t...

Although the gastrointestinal tract is susceptible to the deposition of amyloid materials with systemic disease, localised amyloidosis of the gut is very rare.' Complications associated with amyloidosis of the gastrointestinal tract are uncommon and colonic perforation is exceptionally rare. We report a case ofperforation of the sigmoid colon secondary to localised amyloidosis and review other ...

Primary (AL) amyloidosis is the most common form of systemic amyloidosis seen in current clinical practice. The symptoms of the disease are usually vague, special features are seen in fewer than one fifth of patients, and the combination of organs and systems involved provides a clue for the diagnosis. We describe a patient in whom asymptomatic hepatomegaly, cardiomegaly, hyperlipidaemia and el...

Localized cutaneous nodular amyloidosis (LCNA) is the rarest form of localized cutaneous amyloidosis. In patients with LCNA, local plasma cells secrete immunoglobulin light chains called amyloid L via an unknown mechanism. LCNA has been associated with autoimmune connective tissue diseases such as most commonly Sjögren syndrome. A few reported cases of LCNA are associated with limited systemic ...