Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2019 Apr;47(4):1778-1786.
doi: 10.1177/0300060518814337. Epub 2019 Feb 25.

Multiorgan involvement by amyloid light chain amyloidosis

Guoliang Li  1 , Dan Han  1 , Suhua Wei, Huaiyu Wang, Limei Chen
Affiliations
Case Reports

Multiorgan involvement by amyloid light chain amyloidosis

Guoliang Li et al. J Int Med Res. 2019 Apr.

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 The normal structure of the liver and spleen had been replaced by amyloid deposition.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.

Keywords: Amyloid light chain (AL) amyloidosis; Congo red; amyloid deposition; chemotherapy; multiorgan involvement; splenic rupture.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
Hepatic hematoxylin and eosin staining showed a nodular hepatic structure, arterial wall thickening in the portal area, extensive accumulation of homogeneous substance (amyloid) among the hepatic cord cells, and difficult-to-recognize hepatic sinusoids.
Figure 2.
Figure 2.
Splenic hematoxylin and eosin staining showed that the ultrastructure of the spleen was impaired and that the red pulp and white pulp were diffusely destroyed with extensive accumulation of homogeneous substance (amyloid).
Figure 3.
Figure 3.
Kidney pathology. (a) Congo red stain showed characteristic amyloid deposition in the glomerulus. (b) Oxidized Congo red stain showed amyloid deposition in the glomerulus, indicating amyloid light chain amyloidosis. (c) Periodic acid–Schiff stain revealed amyloid deposition in the glomerulus.
Figure 4.
Figure 4.
(a) Computed tomography showed an enlarged liver mass (red arrow) in accordance with diffuse liver damage. (b) The most recent follow-up computed tomography image showed a diffusely enlarged liver (blue arrow), as before.
See this image and copyright information in PMC

References

    1. Zumbo G, Sadeghi-Alavijeh O, Hawkins PN, et al. New and developing therapies for AL amyloidosis. Expert Opin Pharmacother 2017; 18: 139–149. - PubMed
    1. Zhang C, Huang X, Li J. Light chain amyloidosis: where are the light chains from and how they play their pathogenic role? Blood Rev 2017; 31: 261–270. - PubMed
    1. Yamamoto T, Maeda N, Kawasaki H. Hepatic failure in a case of multiple myeloma-associated amyloidosis (kappa-AL). J Gastroenterol 1995; 30: 393–397. - PubMed
    1. Mahmood S, Bridoux F, Venner CP, et al. Natural history and outcomes in localised immunoglobulin light-chain amyloidosis: a long-term observational study. Lancet Haematol 2015; 2: e241–e250. - PubMed
    1. Oran B, Wright DG, Seldin DC, et al. Spontaneous rupture of the spleen in AL amyloidosis. Am J Hematol 2003; 74: 131–135. - PubMed

Publication types

MeSH terms