Systemic transtiretin amyloidosis in the elderly patient with recurrent pleural effusions

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Abstract

Systemic amyloidosis is a group of diseases associated with extracellular deposition of fibrillar proteins, which leads to a loss of normal organ structure and function. Transthyretin amyloidosis occurs with the deposition of amyloid, consisting of transthyretin transport protein, and can be a genetic or degenerative disease of senility (acquired from the deposition of wild-type transthyretin).

The article describes the clinical case of transthyretine amyloidosis in elderly patient, manifested by recurrent pleural effusions and biventricular heart failure demonstrating the complexity of timely diagnosis of wild-type transthyretin amyloidosis.

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About the authors

Svetlana A. Boldueva

North-Western State Medical University named after I.I. Mechnikov

Email: svetlanaboldueva@mail.ru
ORCID iD: 0000-0002-1898-084X
Scopus Author ID: 6506877971

MD, Dr. Sci. (Med.), Professor

Russian Federation, 41 Kirochnaya St., Saint Petersburg, 191015

Dmitriy S. Evdokimov

North-Western State Medical University named after I.I. Mechnikov

Author for correspondence.
Email: kasabian244@gmail.com
ORCID iD: 0000-0002-3107-1691

аспирант кафедры факультетской терапии

Russian Federation, 41 Kirochnaya St., Saint Petersburg, 191015

Natalia S. Shvets

North-Western State Medical University named after I.I. Mechnikov

Email: natashashvets@yandex.ru

MD, Cand. Sci. (Med.)

Russian Federation, 41 Kirochnaya St., Saint Petersburg, 191015

Anahit V. Shahbazyan

North-Western State Medical University named after I.I. Mechnikov

Email: shahbazyananahit13@gmail.com
ORCID iD: 0000-0002-6923-5321

врач-кардиолог

Russian Federation, 41 Kirochnaya St., Saint Petersburg, 191015

Elena Yu. Kalinina

Saint-Petersburg State Pediatric Medical University

Email: drkalinina@yandex.ru

MD, Cand. Sci. (Med.)

Russian Federation, Saint Petersburg

Lyubov B. Mitrofanova

Almazov National Medical Research Centre

Email: lubamitr@yandex.ru

MD, Dr. Sci. (Med.), Assistant Professor

Russian Federation, Saint Petersburg

References

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  2. Connors LH, Sam F, Skinner M, et al. Heart failure resulting from age-related cardiac amyloid disease associated with wild-type transthyretin: a prospective, observational Cohort Study. Circulation. 2016;133(3):282–290. doi: 10.1161/CIRCULATIONAHA.115.018852
  3. Boldueva SA, Petrova VB, Khavinson VKh, et al. Problems in the diagnostics and treatment of transtiretinum amyloidosis with heart disease in the elderly: clinical experience. Advances in Gerontology. 2019;32(1–2):137–144. (In Russ.)
  4. Castaño A, Drachman BM, Judge D, Maurer MS. Natural history and therapy of TTR-cardiac amyloidosis: emerging disease-modifying therapies from organ transplantation to stabilizer and silencer drugs. Heart Fail Rev. 2015;20(2):163–178. doi: 10.1007/s10741-014-9462-7
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  7. Poliakova AA, Semernin EN, Sitnikova MY, et al. Transthyretin amyloidosis in a cohort fold and very old patients with chronic heart failure. Kardiologiia. 2018;58(S2):12–18. (In Russ.). doi: 10.18087/cardio.2390
  8. Dyudina IA. Transtyretin amyloidosis: the current state of the problem. Heart failure and comorbid conditions.2017;1:63–68. (In Ukraine)
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  10. Fernandes A, Caetano F, Almeida I, et al. Diagnostic approach to cardiac amyloidosis: A case report. Amiloidose cardíaca – a bordagem diagnóstica, a propósito de um casoclínico. Rev Port Cardiol. 2016;35(5):305.e1–305.e7. doi: 10.1016/j.repc.2016.01.004
  11. Lysenko (Kozlovskaya) LV, Rameev VV, Moiseev S, et al. Clinical guidelines for diagnosis and treatment of systemic amyloidosis. Klinicheskaya farmakologiya i terapiya. 2020;(1):13–24.(In Russ.). DOI: 10.32756/ 0869-5490-2020-1-13-24
  12. Berk JL. Pleural effusions in systemic amyloidosis. Curr Opin Pulm Med. 2005;11(4):324–328. doi: 10.1097/01.mcp.0000162378.35928.37
  13. Mansalis KA, Klein DA, Demartini SD, et al. Pleural findings in a patient with persistent pulmonary effusions from systemic amyloidosis. Amyloid. 2011;18(1):29–31. doi: 10.3109/13506129.2010.537156

Supplementary files

Supplementary Files
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2. Fig. 1. The electrocardiogram at the admission

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3. Fig. 2. The echocardiography at the admission

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4. Fig. 3. Chest X-ray data: a — 05.02; b — 11.02; c — 13.02

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5. Fig. 4. Magnification ×400. Congo-red. Deposition of amyloid under the endocardium

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6. Fig. 5. Magnification ×400. Congo-red. Polarized light microscopy. Amyloid in the artery wall

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7. Fig. 6. Magnification ×400. Congo-red. Polarized light microscopy. Myocardium. Perivascular amyloid deposition

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8. Fig. 7. Magnification ×200. Congo-red. Deposition of amyloid in the myocardium

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9. Fig. 8. Magnification ×200. Congo-red. Myocardium. Deposition of amyloid in the stroma

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10. Fig. 9. Magnification ×400. Microscopy in polarized light. Amyloid on the tongue

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11. Fig. 10. Gross specimen of the heart, left ventricular (LV) section. There is a significant thickening of the LV walls, small size of the LV cavity

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Copyright (c) 2021 Boldueva S.A., Evdokimov D.S., Shvets N.S., Shahbazyan A.V., Kalinina E.Y., Mitrofanova L.B.

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This work is licensed under a Creative Commons Attribution 4.0 International License.

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