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HomeArticlesVol 33,2023 No.4Detail

A case of amyloid light amyloidosis involving multiorgan with hepatomegaly as the main manifestation

Published on Aug. 25, 2023Total Views: 6024 timesTotal Downloads: 1648 timesDownloadMobile

Author: Ya-Nan CHEN 1, 2, 3# Jia-Long LIU 1, 2, 3# Yi-Zhang LI 1, 2, 3 Ji-Xia ZHANG 1, 2, 3 Xian-Yan SHI 1, 2, 3 Jing LIU 1, 2, 3

Affiliation: 1. Department of Gastroenterology, Zhongnan Hospital of Wuhan University, Wuhan 430071, China 2. Hubei Clinical Center of Intestinal and Colorectal Diseases, Wuhan 430071, China 3. Hubei Key Lab of Intestinal and Colorectal Diseases, Wuhan 430071, China #Co-first author: Ya-Nan CHEN and Jia-Long LIU

Keywords: Amyloidosis Light chain amyloidosis Multiple organ involvement

DOI: 10.12173/j.issn.1004-5511.202209054

Reference: Chen YN, Liu JL, Li YZ, Zhang JX, Shi XY, Liu J. A case of amyloid light amyloidosis involving multiorgan with hepatomegaly as the main manifestation[J]. Yixue Xinzhi Zazhi, 2023, 33(4): 285-290. DOI: 10.12173/j.issn.1004-5511.202209054.[Article in Chinese]

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Abstract

Amyloid light (AL) amyloidosis, also known as primary systemic amyloidosis, is a protein conformation disease, and is a clinical syndrome of functional failure of affected organs due to abnormal folding and deposition of insoluble fibrin in the extracellular region of organs or tissues. The diagnosis of AL-type amyloidosis is challenging because of its low incidence and complex clinical manifestations, and cases of multiple organ involvement are even rarer. We reported a case of AL-type amyloidosis with multiple organ involvement in order to provide refference for related diagnosis, treatment and prognosis.

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References

Picken MM. The pathology of amyloidosis in classification: a review[J]. Acta Haematol, 2020, 143(4): 322-334. DOI: 10.1159/000506696.

Benson MD, Buxbaum JN, Eisenberg DS, et al. Amyloid nomenclature 2018: recommendations by the International Society of Amyloidosis (ISA) nomenclature committee[J]. Amyloid, 2018, 25(4): 215-219. DOI: 10.1080/13506129.2018.1549825.

Ryšavá R. AL amyloidosis: advances in diagnostics and treatment[J]. Nephrol Dial Transplant, 2019, 34(9): 1460-1466. DOI: 10.1093/ndt/gfy291.

Vaxman I, Gertz M. When to suspect a diagnosis of amyloidosis[J]. Acta Haematol, 2020, 143(4): 304-311. DOI: 10.1159/000506617.

Quock TP, Yan T, Chang E, et al. Epidemiology of AL amyloidosis: a real-world study using US claims data[J]. Blood Adv, 2018, 2(10): 1046-1053. DOI: 10.1182/bloodadvances.2018016402.

Rossi A, Voigtlaender M, Janjetovic S, et al. Mutational landscape reflects the biological continuum of plasma cell dyscrasias[J]. Blood Cancer J, 2017, 7(2): e537. DOI: 10.1038/bcj.2017.19.

Merlini G, Dispenzieri A, Sanchorawala V, et al. Systemic immunoglobulin light chain amyloidosis[J]. Nat Rev Dis Primers, 2018, 4(1): 38. DOI: 10.1038/s41572-018-0034-3.

Li G, Han D, Wei S, et al. Multiorgan involvement by amyloid light chain amyloidosis[J]. J Int Med Res, 2019, 47(4): 1778-1786. DOI: 10.1177/0300060518814337.

Hasib Sidiqi M, Gertz MA. Immunoglobulin light chain amyloidosis diagnosis and treatment algorithm 2021[J]. Blood Cancer J, 2021, 11(5): 90. DOI: 10.1038/s41408-021-00483-7.

Gupta N, Kaur H, Wajid S. Renal amyloidosis: an update on diagnosis and pathogenesis[J]. Protoplasma, 2020, 257(5):1259-1276. DOI: 10.1007/s00709-020-01513-0.

Oghina S, Delbarre MA, Poullot E, et al. [Cardiac amyloidosis: state of art in 2022][J]. Rev Med Interne, 2022, 43(9): 537-544. DOI: 10.1016/j.revmed.2022.04.036.

Talar-Wojnarowska R, Jamroziak K. Intestinal amyloidosis: Clinical manifestations and diagnostic challenge[J]. Adv Clin Exp Med, 2021, 30(5): 563-570. DOI: 10.17219/acem/133521.

Rosenzweig M, Kastritis E. Liver and gastrointestinal involvement: update[J]. Hematol Oncol Clin North Am, 2020, 34(6s):e1-e13. DOI: 10.1016/j.hoc.2020.11.001.

Kaku M, Berk JL. Neuropathy associated with systemic amyloidosis[J]. Semin Neurol, 2019, 39(5): 578-588. DOI: 10.1055/s-0039-1688994.

Desai SS, Rizzo MG, Rush AJ 3rd, et al. Amyloidoma: a review and case report[J]. Skeletal Radiol, 2021, 50(2): 437-444. DOI: 10.1007/s00256-020-03560-3.

Gertz MA. Immunoglobulin light chain amyloidosis: 2022 update on diagnosis, prognosis, and treatment[J]. Am J Hematol, 2022, 97(6): 818-829. DOI: 10.1002/ajh.26569.

Gertz MA. Immunoglobulin light chain amyloidosis: 2020 update on diagnosis, prognosis, and treatment[J]. Am J Hematol, 2020, 95(7): 848-860. DOI: 10.1002/ajh.25819.

Wechalekar AD, Gillmore JD, Hawkins PN. Systemic amyloidosis[J]. Lancet, 2016, 387(10038): 2641-2654. DOI: 10.1016/S0140-6736(15)01274-X.

Palladini G, Milani P, Merlini G. Management of AL amyloidosis in 2020[J]. Hematology Am Soc Hematol Educ Program, 2020, 2020(1):363-371. DOI: 10.1182/hematology.2020006913.

Milani P, Palladini G. Conventional therapy for amyloid light-chain amyloidosis[J]. Acta Haematol, 2020, 143(4): 365-372. DOI: 10.1159/000507072.

Desport E, Bridoux F, Sirac C, et al. Al amyloidosis[J]. Orphanet J Rare Dis, 2012, 7: 54. DOI: 10.1186/1750-1172-7-54.

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