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DOI: https://doi.org/10.22263/2312-4156.2024.2.69

A.A. Dyachkova, L.A. Avtaykina
Primary AL amyloidosis (Lambda chain ++) with the damage to the kidneys, heart, thyroid gland (clinical case)
National Research Mordovian State University named after N.P. Ogarev, Saransk, Russian Federation

Vestnik VGMU. 2024;23(2):69-76.

Abstract.
Primary amyloidosis is characterized by a varied clinical picture, but predominantly affects the kidneys and the heart. The debut of this pathology can be disguised as the initial manifestations of other diseases, in connection with this, patients see specialists of various profiles (a therapist, a cardiologist, etc.), and not a rheumatologist, whose competence is the diagnosis and treatment of the disease.
This article presents the analysis of a clinical case of primary amyloidosis (AL type) with predominant damage to the kidneys, the heart and thyroid gland in a 66-year-old woman. This patient had a clinical picture characteristic of amyloidosis and changes while using laboratory and instrumental investigation methods. However, the diagnostic search was complicated by cardiac damage masking itself as hypertensive cardiomyopathy, which led to the erroneous assumption of the presence of a variant of amyloidosis associated with multiple myeloma due to the existing signs of nephropathy. Timely diagnosis and administration of rational treatment resulted in a decrease of the pathological process activity and stabilization of the patient’s condition.
The complexity of the diagnostic search in this clinical case indicates the need for increased attention of practicing physicians and requires an individual approach to the management of each patient in order to minimize making an erroneous diagnosis and reducing the volume of additional examination methods.
Keywords: AL amyloidosis, primary amyloidosis, restrictive cardiomyopathy.

References

1. Blagova OV, Nedostup AV, Sedov VP, Kogan EA, Pasha SP, Gagarina NV, i dr. Clinical masks of amyloidosis with cardiac lesions: peculiarities of diagnostics at the present stage. Ros Kardiol Zhurn. 2017;(2):68-79. (In Russ.)
2. Dovidchenko NV, Leonova EI, Galzitskaya OV. Mechanisms of amyloid fibril formation. Uspekhi Biol Khimii. 2014;54:203-30. (In Russ.)
3. Sharma A, Govindan P, Toukatly M, Healy J, Henry C, Senter S, et al. Heroin Use Is Associated with AA–Type Kidney Amyloi-dosis in the Pacific Northwest. Clin J Am Soc Nephrol. 2018 Jul;13(7):1030-6. doi: http://dx.doi.org/10.2215/CJN.13641217
4. Gudkova AYa, Lapekin SV, Bezhanishvili TG, Trukshina MA, Davydova VG, Krutikov AN, i dr. AL-amyloidosis with predominant cardiac involvement. Algorithm of non-invasive diagnosis of amyloid cardiomyopathy. Terapevt Arkh. 2021;93(4):487-96. (In Russ.). doi: http://dx.doi.org/10.26442/00403660.2021.04.200689
5. Cherkasova NA, Komarova IS, Mukhina NV, Dyatlov NV. Clinical case of primary generalized amyloidosis. Klin Razbor Obshchei Meditsine. 2023;4(2):27-30. (In Russ.). doi: http://dx.doi.org/10.47407/kr2023.4.2.00200
6. Gomzikova EA, Samsonova MV, Chernyaev AL, Kurkov AV. Pulmonary amyloidosis: basics of diagnosis. Prakt Pul'monologiya. 2017;(3):90-8. (In Russ.)
7. Anikonova LI, Vorobyeva OA, Bakulina NV. ATTR amyloidosis is a systemic disease with renal involvement. Nefrologiya Dializ. 2022;24(3):441-56. (In Russ.). doi: http://dx.doi.org/10.28996/2618-9801-2022-3-441-456
8. Aksenova ME, Dlin VV, Turpitko OYu, Nevstrueva VV. Amyloid nephropathy in a child with intermittent disease. Praktika Pediatra. 2015;(4):358-92. (In Russ.)
9. Lysenko (Kozlovskaya) LV, Rameev VV, Moiseev SV, Blagova OV, Bogdanov EI, Gendlin GE, i dr. Clinical guidelines for the diagnosis and treatment of systemic amyloidosis. Klin Farmakologiya Terapiya. 2020;29(1):13-24. (In Russ.). doi: http://dx.doi.org/10.32756/ 0869-5490-2020-1-13-24
10. Stepanova EA. Possibilities of morphologic diagnosis of amyloid in biopsy specimens of subcutaneous adipose tissue. Arkh Patologii. 2022;84(1):14-20. (In Russ.). doi: http://dx.doi.org/10.17116/patol20228401114
11. Reznik EV, Nguen TL, Stepanova EA, Ustyuzhanin DV, Nikitin IG. Cardiac amyloidosis: an internist and cardiologist's perspective. Arkh Vnutr Meditsiny. 2020;10(6):430-57. (In Russ.). doi: http://dx.doi.org/10.20514/2226-6704-2020-10-6-430-457
12. Reznik EV, Nguen TL, Ustyuzhanin DV, Semyachkina AN, Shkolnikova MA. «Red flags» of the diagnosis of infiltrative heart disease. Ros Kardiol Zhurn. 2023;28(1S):52-9. (In Russ.). doi: http://dx.doi.org/10.15829/1560-4071-2023-5259
13. Boldueva SA, Oblavatskiy DV, Grokhotova VV, Bystrova OB, Mayer DA, Dobronravov VA. Clinical observation of systemic AL amyloidosis with unusual disease debut. Nefrologiya. 2017;21(6):78-85. (In Russ.). doi: http://dx.doi.org/10.24884/1561-6274-2017-21-6-78-85
14. Borisenko AA, Kim EI, Pupysheva AD, Dmitrieva KV, Smorodina AS. Review of rare clinical cases. Sinergiya Nauk. 2018;(26):520-5. (In Russ.)
15. Smirnova EA, Abdurakhmanova EK, Filonenko SP. Systemic AL amyloidosis: difficulties in diagnosis (literature review and own data). Ros Med-biol Vestn im akad IP Pavlova. 2016;24(3):141-53. (In Russ.). doi: http://dx.doi.org/10.17816/PAVLOVJ20163141-153
16. Mendeleeva LP, Votyakova OM, Pokrovskaya OS, Rekhtina IG, Bessmeltsev SS, Golubeva ME. National Clinical Guidelines for the Diagnosis and Treatment of Multiple Myeloma. Gematologiya Transfuziologiya. 2014;59(1 прил 3):3-24. (In Russ.)

Information about authors:
A.A. Dyachkova – Candidate of Medical Sciences, associate professor of the Chair of Faculty Therapy with the course of Medical Rehabilitation of the Medical Institute, National Research Mordovian State University named after N.P. Ogarev, https://orcid.org/0000-0002-4596-2970
L.A. Avtaykina – the fourth-year student of the specialty “General Medicine” of the Medical Institute, National Research Mordovian State University named after N.P. Ogarev, https://orcid.org/0009-0002-3473-5200
e-mail: Этот адрес электронной почты защищён от спам-ботов. У вас должен быть включен JavaScript для просмотра. – Lyubov A. Avtaykina.

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