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.
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.
Graduate Medical Sciences Division, Boston University School of Medicine, Boston, Massachusetts, USA.
Martin
M
Tam
David C
DC
Seldin
Benjamin M
BM
Forbes
Lawreen H
LH
Connors
Martha
M
Skinner
Betul
B
Oran
Karen
K
Quillen
Vaishali
V
Sanchorawala
D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000150:complications* / Q000378:metabolism; D004591:Electrophoresis, Polyacrylamide Gel; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008107:Liver Diseases / Q000601:surgery*; D008558:Melphalan / Q000008:administration & dosage*; D008875:Middle Aged; D012422:Rupture, Spontaneous; D033581:Stem Cell Transplantation; D019172:Transplantation Conditioning
D000682:Amyloid; D008558:Melphalan