Multiorgan involvement by amyloid light chain amyloidosis

The Journal of International Medical Research
Guoliang LiLimei Chen

Abstract

Amyloid light chain (AL) amyloidosis is a protein conformational disease. AL amyloidosis results from aggregation of misfolded proteins that are deposited in tissues as amyloid fibrils. Diagnosis of AL amyloidosis can be challenging due to its low incidence and clinical complexity. Therapy requires a risk-adapted approach involving dose reductions and schedule modifications of chemotherapy regimens along with close monitoring of hematologic and organ responses. We herein describe a patient whose condition was diagnosed as systemic AL amyloidosis and presented with splenic rupture as the initial symptom. Congo red staining of the kidney biopsy was positive. The normal structure of the liver and spleen had been replaced by amyloid deposition. The chemotherapy strategy involved a combination of bortezomib, cyclophosphamide, thalidomide, and dexamethasone.

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Citations

Jul 30, 2020·The Journal of Hand Surgery Asian-Pacific Volume·Yukinori HaraYusuke Kita
Jul 16, 2020·Case Reports in Surgery·Kathrin DvirKfir Ben-David
Jan 26, 2021·The American Journal of Dermatopathology·Ogochukwu Nefertiti UmehBen J Friedman
Feb 3, 2021·European Journal of Clinical Investigation·Thamonwan DiteepengMarco Luciani
Oct 17, 2021·Digestive Diseases and Sciences·Nilofar NajafianGina Choi

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Methods Mentioned

BETA
biopsy
laser capture microdissection
biopsies

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