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Ledesma FL, Castelli JB. Autopsy findings in a patient with primary systemic AL (kappa light chain) amyloidosis. Autops Case Rep. 2021;11:e2021273doi: 10.4322/acr.2021.273.
Ledesma, F. L., & Castelli, J. B. (2021). Autopsy findings in a patient with primary systemic AL (kappa light chain) amyloidosis. Autopsy & case reports, 11e2021273. https://doi.org/10.4322/acr.2021.273
Ledesma, Felipe Lourenço, and Castelli, Jussara Bianchi. "Autopsy findings in a patient with primary systemic AL (kappa light chain) amyloidosis." Autopsy & case reports vol. 11 (2021): e2021273. doi: https://doi.org/10.4322/acr.2021.273
Ledesma FL, Castelli JB. Autopsy findings in a patient with primary systemic AL (kappa light chain) amyloidosis. Autops Case Rep. 2021 May 06;11:e2021273. doi: 10.4322/acr.2021.273. eCollection 2021. PMID: 34307229; PMCID: PMC8214892.
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Autops Case Rep. 2021 May 06;11:e2021273. doi: 10.4322/acr.2021.273. eCollection 2021.

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

Autopsy & case reports

Felipe Lourenço Ledesma, Jussara Bianchi Castelli

Affiliations

  1. Universidade de São Paulo (USP), Faculdade de Medicina, Departamento de Patologia, São Paulo, SP, Brasil.
  2. Universidade de São Paulo (USP), Hospital das Clínicas, Divisão de Anatomia Patológica, São Paulo, SP, Brasil.
  3. Grupo Fleury Medicina e Saúde, São Paulo, SP, Brasil.

PMID: 34307229 PMCID: PMC8214892 DOI: 10.4322/acr.2021.273

Abstract

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.

Copyright: © 2021 The Authors.

Keywords: Amyloidosis; Autopsy; Diagnosis; Multiple Organ Failure; Pneumatosis Cystoides Intestinalis; Tandem Mass Spectrometry

Conflict of interest statement

Conflict of interest: None.

References

  1. N Engl J Med. 1980 Jun 5;302(23):1283-92 - PubMed
  2. Cent Eur J Immunol. 2014;39(1):61-6 - PubMed
  3. Thyroid. 2001 Apr;11(4):397-400 - PubMed
  4. Amyloid. 2020 Dec;27(4):217-222 - PubMed
  5. Blood. 2015 Apr 2;125(14):2239-44 - PubMed
  6. J Clin Gastroenterol. 1996 Jan;22(1):74-6 - PubMed
  7. Semin Hematol. 1995 Jan;32(1):45-59 - PubMed
  8. Am J Med. 1962 Sep;33:349-57 - PubMed
  9. Hematol Oncol Clin North Am. 1999 Dec;13(6):1211-33, ix - PubMed
  10. Int J Dermatol. 2019 Sep;58(9):1062-1068 - PubMed
  11. Haematologica. 2000 Mar;85(3):289-92 - PubMed
  12. J Pathol. 1987 Jan;151(1):1-10 - PubMed
  13. J Clin Med Res. 2017 Jul;9(7):654-658 - PubMed
  14. Clin Lymphoma Myeloma Leuk. 2013 Apr;13(2):244-6 - PubMed
  15. Clin Proteomics. 2016 Oct 27;13:30 - PubMed
  16. J Clin Gastroenterol. 2008 Jul;42(6):758-9 - PubMed
  17. Arch Intern Med. 1988 Apr;148(4):988 - PubMed
  18. Medicine (Baltimore). 1975 Jul;54(4):271-99 - PubMed
  19. Arch Intern Med. 1988 Apr;148(4):929-33 - PubMed
  20. Hippokratia. 2008;12(4):236-9 - PubMed
  21. Nephrol Dial Transplant. 2009 Oct;24(10):3132-7 - PubMed
  22. J Pathol Clin Res. 2019 Jul;5(3):145-153 - PubMed
  23. Am J Clin Pathol. 1995 Sep;104(3):306-12 - PubMed
  24. Am J Gastroenterol. 2008 Mar;103(3):776-87 - PubMed
  25. Gastrointest Endosc. 1990 Jan-Feb;36(1):10-4 - PubMed
  26. Prz Gastroenterol. 2014;9(1):57-61 - PubMed
  27. Arthritis Rheum. 1989 Jan;32(1):82-5 - PubMed
  28. Am J Med. 2015 Sep;128(9):e3-4 - PubMed
  29. Ann Saudi Med. 2009 Mar-Apr;29(2):138-41 - PubMed
  30. Am J Clin Pathol. 1961 Oct;36:341-55 - PubMed
  31. Postgrad Med J. 1982 Mar;58(677):171-2 - PubMed
  32. Autops Case Rep. 2016 Jun 30;6(2):9-18 - PubMed
  33. Arthritis Rheum. 2006 Jun;54(6):2015-21 - PubMed
  34. Heart. 2011 Jan;97(1):75-84 - PubMed
  35. Int J Surg Case Rep. 2016;23:29-32 - PubMed
  36. Semin Nephrol. 2002 Jul;22(4):319-30 - PubMed
  37. Case Rep Gastroenterol. 2013 Dec 12;7(3):511-5 - PubMed

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