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Case Report
Systemic AL amyloidosis presenting as progressive hepatic failure with coagulopathy: An autopsy case report
1 Department of Pathology & Immunology, Baylor College of Medicine, Houston, TX 77030, United States
2 Department of Pathology, Ben Taub General Hospital, Houston, TX 77030, United States
Address correspondence to:
Ya Xu
MD, PhD, Department of Pathology & Immunology, Baylor College of Medicine, BCM 315, One Baylor Plaza, Houston, TX 77030,
United States
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Article ID: 101161Z01LS2020
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Suo L, Mousavi S, Ward TL, Xu Y. Systemic AL amyloidosis presenting as progressive hepatic failure with coagulopathy: An autopsy case report. Int J Case Rep Images 2020;11:101161Z01LS2020.ABSTRACT
Introduction: Systemic AL amyloidosis is defined as extracellular deposition of insoluble immunoglobulin light chains, amyloid fibril protein AL, in at least one vital organ. Here we report a case of systemic AL amyloidosis presenting as progressive hepatic failure with coagulopathy.
Case Report: The patient was a 62-year-old African American female first admitted to our hospital due to jaundice, easy bruising, fatigue, and weight loss for three months. There were mild abnormal liver function tests with coagulopathy, polyclonal gammopathy, and mild hepatomegaly by imaging. She was discharged after correction of coagulopathy. She presented with altered mental status one month later. Lab results showed similar abnormal liver function tests and hepatomegaly but with severe coagulopathy. The patient was found to have oozing blood, severe hypoxia, and expired the next day. A complete autopsy revealed systemic amyloidosis microscopically, and confirmed by Congo red stain. The liver demonstrated the most extensive deposition of amyloid. Liquid chromatography tandem mass spectrometry revealed AL (kappa)-type amyloid deposition. Bone marrow showed kappa light chain predominance.
Conclusion: Systemic amyloidosis presenting as progressive hepatic failure is very rare. Amyloidosis with liver involvement is one of the important differential diagnoses even when patients present with mild liver dysfunction (especially elevated alkaline phosphatase and gamma-glutamyl transerase) and coagulopathy.
Keywords: Coagulopathy, Hepatic failure, Systemic AL amyloidosis
SUPPORTING INFORMATION
Author Contributions
Liye Suo - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Shima Mousavi - Substantial contributions to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Tabitha Lynn Ward - Acquisition of data, Analysis of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Ya Xu - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guaranter of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient’s next of kin for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2020 Liye Suo et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
