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Amyloidosis of the gastrointestinal tract: a 13-year single center referral experience.
MedLine Citation:
PMID:  22733017     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Background. Amyloidosis of the gastrointestinal tract, with biopsy-proven disease, is rare. We review a series of patients who presented with biopsy proven gastrointestinal amyloidosis and report clinical characteristics, treatments, and survival. Design and Methods This is a retrospective review of data prospectively collected from January 1998 to December 2011 in a tertiary referral center; 2,334 patients with all types of amyloidosis were evaluated during this time. Results. 76 patients (3.2%) had biopsy-proven amyloid involvement of the gastrointestinal tract. The median age was 61 years (range 34 - 79). Systemic amyloidosis with dominant gastrointestinal involvement was present in 60 (79%) patients. Amyloidosis localized to the gastrointestinal tract without evidence of an associated plasma cell dyscrasia or other organ involvement comprised 16 (21%) patients. Of the 60 systemic cases, 50 (83%) had immunoglobulin light-chain, 5 (8%) familial lysozyme, 3 (5%) wild type transthyretin, and 2 (3%) mutant transthyretin amyloidosis. The most frequent symptoms for all patients were weight loss in 33 (45%) and gastrointestinal bleeding in 27 (36%). Incidental identification of amyloidosis on routine endoscopic surveillance played a role in the diagnosis of 7 patients with systemic immunoglobulin light-chain, and 4 patients with immunoglobulin light-chain localized to the gastrointestinal tract. Amyloid protein subtyping was performed in 12 of the cases of localized disease, and all had lambda light chain disease. Of the 50 patients with systemic immunoglobulin light-chain amyloidosis, 45 patients were treated with anti-plasma cell therapy. The median survival has not been reached for this group. For the 16 patients with localized gastrointestinal amyloidosis, supportive care was the mainstay of treatment; none received anti plasma cell therapy. All 16 are alive at a median follow-up of 36 months (range 1-143). Conclusions. Patients with biopsy-proven gastrointestinal amyloidosis often present with weight loss and bleeding. In localized cases, all that underwent typing were due to λ light chain amyloidosis and none progressed to systemic disease during the period of follow-up. Most patients with systemic disease had immunoglobulin light-chain, and their tolerance of therapy and median survival were excellent. Although a rare manifestation of amyloidosis, staining for amyloid should be considered in patients undergoing gastrointestinal biopsy who have unexplained chronic gastrointestinal symptoms.
Authors:
Andrew J Cowan; Martha Skinner; David C Seldin; John L Berk; David R Lichtenstein; Carl J O' Hara; Gheorghe Doros; Vaishali Sanchorawala
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-6-24
Journal Detail:
Title:  Haematologica     Volume:  -     ISSN:  1592-8721     ISO Abbreviation:  -     Publication Date:  2012 Jun 
Date Detail:
Created Date:  2012-6-26     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0417435     Medline TA:  Haematologica     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
USA.
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