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    
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.
Martin Tam; David C Seldin; Benjamin M Forbes; Lawreen H Connors; Martha Skinner; Betul Oran; Karen Quillen; Vaishali Sanchorawala
Related Documents :
1995384 - Group b streptococcal chorioamnionitis and neonatal septicemia following 8 days pivampi...
22215504 - Role of primary bacterial contamination of a pulmonary homograft for ross operation: re...
16874704 - Spontaneous subcapsular hematoma in chronic kidney disease.
18988934 - Case report: spontaneous splenic rupture during acute parasitemia of babesia microti.
24381794 - Analysis of a synchronous gliosarcoma and meningioma with long survival: a case report ...
19097384 - Ozone and its usage in general medicine and dentistry. a review article.
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    
Graduate Medical Sciences Division, Boston University School of Medicine, Boston, Massachusetts, USA.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Amyloid / metabolism
Amyloidosis / complications*,  metabolism
Electrophoresis, Polyacrylamide Gel
Fatal Outcome
Liver Diseases / surgery*
Melphalan / administration & dosage*
Middle Aged
Rupture, Spontaneous
Stem Cell Transplantation*
Transplantation Conditioning*
Grant Support
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
Reg. No./Substance:
0/Amyloid; 148-82-3/Melphalan

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

Previous Document:  Cardiomyopathy in a Japanese family with the Glu61Lys transthyretin variant: a new phenotype.
Next Document:  Adsorption of Bence-Jones protein to polymethylmethacrylate membrane in primary amyloidosis.