Autopsy findings in a patient with primary systemic AL (Kappa light chain) amyloidosis

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art_LEDESMA_Autopsy_findings_in_a_patient_with_primary_systemic_2021.PDF (4.2 MB)
Citações na Scopus
4
Tipo de produção
article
Data de publicação
2021
DOI
Scopus
Título da Revista
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Editora
HOSPITAL UNIVERSITARIO DA UNIVERSIDADE DE SAO PAULO
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Citação
AUTOPSY AND CASE REPORTS, v.11, article ID e2021273, p, 2021
Projetos de Pesquisa
Unidades Organizacionais
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Resumo
First described by Rokitansky in 1842, and further characterized by Virchow in 1854, amyloidosis is a disorder caused by amyloid deposition, a fibrillary insoluble protein. The clinical spectrum of amyloidosis is broad, as the amyloid deposition may virtually occur in all tissues. Herein, we report the case of a 66-year-old man with a long-lasting emaciating disease, diagnosed, at autopsy, with primary systemic amyloidosis. Amyloid protein deposition was found in many tissues and organs. The involvement of the vessels’ wall rendered ischemic injury most prominent in the intestinal loops causing mesenteric ischemia. Despite the thorough organic involvement, the immediate cause of death was aspiration bronchopneumonia. Massive amyloid deposition was found in virtually all major organs, such as the heart, liver, kidneys, spleen, pancreas, adrenals, prostate, skin, and thyroid: the latter, a complication of the amyloidosis known as amyloid goiter. Post-mortem review of the deceased’s laboratory workup showed a slightly abnormal kappa:lambda ratio in the blood; however, no clonal lymphoplasmacytic disorder was confirmed in the bone marrow and other lymphoreticular system organs either by the microscopic examination and immunohistochemical staining. Laser-capture microdissection and tandem mass spectrometry of the splenic tissue detected a peptide profile consistent with an immunoglobulin Kappa light chain. The presence of amyloid purpura favors the diagnosis of primary systemic amyloidosis. © 2021 The Authors.
Palavras-chave
Amyloidosis, Autopsy, Diagnosis, Multiple organ failure, Pneumatosis cystoides intestinalis, Tandem mass spectrometry
URI
https://observatorio.fm.usp.br/handle/OPI/44939
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Artigos e Materiais de Revistas Científicas - HC/InCor
Artigos e Materiais de Revistas Científicas - HC/ICHC