AI Article Synopsis
- A 55-year-old woman with AL systemic amyloidosis died from a spontaneous liver rupture after undergoing high-dose melphalan and autologous stem cell transplant (HDM/SCT).
- She developed nephrotic-range proteinuria, and the rupture occurred 10 days post-treatment, leading to septic shock and ultimately her death.
- The autopsy revealed liver amyloid fibrils made of non-glycosylated immunoglobulin lambda light chains, marking this as the first recorded case of spontaneous liver rupture in a patient undergoing HDM/SCT treatment for amyloidosis.
Article Abstract
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.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2911629 | PMC |
| http://dx.doi.org/10.1080/13506120902879574 | DOI Listing |
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