Document Detail


Spontaneous rupture of the liver in a patient with systemic AL amyloidosis undergoing treatment with high-dose melphalan and autologous stem cell transplantation: a case report with literature review.
MedLine Citation:
PMID:  20536404     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A 55-year-old woman with primary Immunoglobulin light chain (AL) systemic amyloidosis died due to spontaneous rupture of her liver following treatment with high-dose melphalan and autologous stem cell transplant (HDM/SCT). She was first diagnosed after developing nephrotic-range proteinuria. Spontaneous rupture of her liver occurred 10 days after treatment with HDM/SCT and was complicated by septic shock. She was not eligible for surgical intervention and died shortly after. Amyloid fibrils were extracted from the autopsied liver sample (05-135L) and the biochemical nature of the fibrils was analyzed using electrophoretic and immunohistochemical techniques. Our testing showed that the fibrils were composed of immunoglobulin lambda light chains that were not glycosylated. While the liver is often involved in AL amyloidosis, this is the first documented case of a spontaneous hepatic rupture in a patient during treatment with HDM/SCT. A literature review of spontaneous liver rupture in patients with amyloidosis is presented.
Authors:
Martin Tam; David C Seldin; Benjamin M Forbes; Lawreen H Connors; Martha Skinner; Betul Oran; Karen Quillen; Vaishali Sanchorawala
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis     Volume:  16     ISSN:  1744-2818     ISO Abbreviation:  Amyloid     Publication Date:  2009  
Date Detail:
Created Date:  2010-06-11     Completed Date:  2010-09-13     Revised Date:  2013-05-29    
Medline Journal Info:
Nlm Unique ID:  9433802     Medline TA:  Amyloid     Country:  England    
Other Details:
Languages:  eng     Pagination:  103-7     Citation Subset:  IM    
Affiliation:
Graduate Medical Sciences Division, Boston University School of Medicine, Boston, Massachusetts, USA.
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MeSH Terms
Descriptor/Qualifier:
Amyloid / metabolism
Amyloidosis / complications*,  metabolism
Electrophoresis, Polyacrylamide Gel
Fatal Outcome
Female
Humans
Immunohistochemistry
Liver Diseases / surgery*
Melphalan / administration & dosage*
Middle Aged
Rupture, Spontaneous
Stem Cell Transplantation*
Transplantation Conditioning*
Grant Support
ID/Acronym/Agency:
P01 HL068705-01/HL/NHLBI NIH HHS; P01 HL068705-010003/HL/NHLBI NIH HHS; P01 HL068705-02/HL/NHLBI NIH HHS; P01 HL068705-020003/HL/NHLBI NIH HHS; P01 HL068705-030003/HL/NHLBI NIH HHS; P01 HL068705-040003/HL/NHLBI NIH HHS; P01 HL068705-050003/HL/NHLBI NIH HHS; P01 HL68705/HL/NHLBI NIH HHS
Chemical
Reg. No./Substance:
0/Amyloid; 148-82-3/Melphalan
Comments/Corrections

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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