Symptomatic primary (amyloid light-chain or AL) amyloidosis of the gastrointestinal (GI) tract is very rare. Most of the patients with symptomatic involvement of the GI tract present with altered motility, malabsorption, or bleeding. We report a case of gastric and colonic amyloidosis on anticoagulation presenting with massive upper and lower GI bleeding. A 67-year-old lady known to have multip...

Amyloid fibrils deposited in secondary amyloidosis contain a unique protein (AA) as the major constituent (1-3). An antigenically related serum protein (SAA) (4, 5), present in normal sera in low concentration (6), is elevated in amyloidosis and in diseases which predispose to amyloidosis, such as rheumatoid arthritis, leprosy, and tuberculosis (4-8). The finding that SAA has a monomeric form a...

SIR, We read with interest the report by Scheinberg et al.' of DMSO and colchicine therapy in amyloid disease. The beneficial effects of colchicine on systemic amyloidosis have been suggested by other investigators.2We also have investigated the effect of colchicine on patients with primary (five patients), reactive (three patients), and familial Mediterranean fever (FMF) related secondary amyl...

Current therapy of primary systemic (AL) amyloidosis with oral melphalan and prednisone remains unsatisfactory, with a median survival of only 13 months. Between 1996 and 2003, 93 patients with biopsyproven AL amyloidosis were enrolled in a prospective US national cooperative group trial. Treatment schema consisted of induction therapy with pulse dexamethasone (DEX), followed by maintenance the...

Hereditary gelsolin amyloidosis (AGel amyloidosis) is an autosomal dominant form of systemic amyloidosis caused by a c.640G>A or c.640G>T mutation in the gene coding for gelsolin. Principal clinical manifestations include corneal lattice dystrophy, cranial neuropathy and cutis laxa with vascular fragility. Signs of minor CNS involvement have also been observed, possibly related to cerebral amyl...

BACKGROUND Amyloidosis is a multisystem disease, and can present with multitude of nonspecific symptoms. Gastrointestinal amyloidosis is common, and gastrointestinal (GI) bleeding in these patients has a wide differential diagnosis. The present case features the distinctive endoscopic finding of submucosal hematoma as a clue to immunoglobin light chain (AL) amyloid involvement of the gastrointe...

In order to classify the underlying disorder in four patients with biopsy-proven amyloidosis without overt monoclonal gammopathy, the cytoplasmic immunoglobulin (Ig) distribution of bone marrow plasma cells was evaluated using direct immunofluorescence microscopy. This procedure revealed the presence of a monoclonal proliferation of light chain containing plasma cells, and thus led to the diagn...

A 54-year-old Japanese man presented with recurrent abdominal pain, fever lasting >5 days, and renal failure. AA amyloidosis was proven by renal and gastric biopsy. Symptoms subsided with the administration of colchicine, but a subsequent recurrence of symptoms did not respond to colchicine. Mediterranean fever gene (MEFV) analysis showed that he was heterozygous for mutations in exon 2 (E148Q/...