Hepatopathy as the first manifestation of systemic AL amyloidosis

Česká verzia

Authors: Fusková J.; Miča T.
Authors place of work: Gastroenterologická ambulance, Nemocnice ATLAS, a. s., Zlín
Published in the journal: Gastroent Hepatol 2018; 72(6): 508-511
Category: Hepatologie: kazuistika
doi: https://doi.org/10.14735/amgh2018508

Summary

Amyloidosis is considered a rare disease characterized by the deposition of insoluble protein – amyloid, into the extracellular space of tissues, which damages their functions. There are about 30 types of amyloidosis, of which the most common is systemic AL amyloidosis, which we include among monoclonal gammopathies. At first, symptoms may be nonspecific, such as fatigue, loss of appetite, and weight loss, or there may be symptoms of damage to specific organs, such as the heart and kidneys and nervous system, but also liver, soft tissue, or GIT. In our case, we document a patient who first presented at our gastroenterology clinic. It is important to consider the possibility of amyloidosis after all other common hepatopathies have been excluded. The diagnosis of this patient was prolonged for several months mainly because the patient hesitated to undergo a liver biopsy, which afterwards clearly confirmed the diagnosis. The patient’s status temporarily improved when symptomatic therapy was implemented. After a targeted examination, damage to other organs was also detected, but the treatment no longer had any influence on the infaust prognosis of the patient. Early diagnosis and treatment is important for amyloidosis, because it can have a significant impact on patient outcome. The problem is that, even with clear indications, this disease is often neglected in diagnostic reports.

Key words:

amyloidosis – hepatomegaly – biopsy

Submitted: 2. 5. 2018

Accepted: 17. 9. 2018

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE „uniform requirements“ for biomedical papers.

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