MULTIPLE MYELOMA AND OTHER PLASMA CELL DISEASES

multiplemyelomawos

AL AMYLOIDOSIS, DIAGNOSIS,RISK ASSESSMENT AND MANAGEMENT

Öznur Aydın

Samsun University, Faculty of Medicine, Department of Hematology, Samsun, Türkiye

Aydın Ö. AL Amyloidosis, Diagnosis, Risk Assessment and Management. In: Sevindik ÖG, editor. Multiple Myeloma and Other Plasma Cell Dyscrasias. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.107-111.

ABSTRACT

AL amyloidosis is a plasma cell dyscrasia resulting from the accumulation of a protein derived from immunoglobulin light chain fragments. It is a rare disease, and its exact prevalence is unknown. Diagnosis requires demonstrating amyloid fibrils in a histological evaluation of an affected organ (e.g., kidney, liver) or an alternative site (e.g., abdominal fat tissue, bone marrow). Treatment depends on the extent of organ involvement. Patients are monitored at regular intervals to assess if the disease is responding to treatment and to determine any necessary changes in management. Prognosis is poor if detected at an advanced stage, with median survival as short as four to six months; cardiac or hepatic failure and infection are the leading causes of death.

Keywords: Amyloidosis; Paraproteinemias; Hematopoietic stem cell transplantation; Pro-brain natriuretic peptide; Mass spectrometry.

Referanslar

  1. Kyle RA, Linos A, Beard CM, Linke RP, Gertz MA, O'Fallon WM, et al. Incidence and natural history of primary systemic amyloidosis in Olmsted County, Minnesota, 1950 through 1989. Blood. 1992 Apr 1;79(7):1817-22. [Crossref]  [PubMed]
  2. Merlini G, Palladini G. Two-hit strategy for treating AL amyloidosis? Blood. 2021 Dec 23;138(25):2596-2598. [Crossref]  [PubMed]
  3. Guijarro-Martínez R, Miragall Alba L, Villar Puchades R, Marqués Mateo M, Puche Torres M, Iglesias Gimilio ME, et al. Rational management of macroglossia due to acquired systemic amyloidosis: does surgery play a role? J Oral Maxillofac Surg. 2009 Sep;67(9):2013-7. [Crossref]  [PubMed]
  4. Rajkumar SV, Dimopoulos MA, Palumbo A, Blade J, Merlini G, Mateos MV et al. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol. 2014;15(12):e538-48. [Crossref]  [PubMed]
  5. Dispenzieri A, Gertz MA, Kyle RA, Lacy MQ, Burritt MF, Therneau TM, et al. Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis. J Clin Oncol. 2004 Sep 15;22(18):3751-7. [Crossref]  [PubMed]
  6. Sanchorawala V, Boccadoro M, Gertz M, Hegenbart U, Kastritis E, Landau H, et al. Guidelines for high dose chemotherapy and stem cell transplantation for systemic AL amyloidosis: EHA-ISA working group guidelines. Amyloid. 2022 Mar;29(1):1-7. [Crossref]  [PubMed]
  7. Kastritis E, Palladini G, Minnema MC, Wechalekar AD, Jaccard A, Lee HC, et al. ANDROMEDA Trial Investigators. Daratumumab-Based Treatment for Immunoglobulin Light-Chain Amyloidosis. N Engl J Med. 2021 Jul 1;385(1):46-58.
  8. Palladini G, Milani P. Diagnosis and Treatment of AL Amyloidosis. Drugs. 2023 Feb;83(3):203-216. [Crossref]  [PubMed]
  9. Kastritis E, Leleu X, Arnulf B, Zamagni E, Cibeira MT, Kwok F,et al. Bortezomib, Melphalan, and Dexamethasone for Light-Chain Amyloidosis. J Clin Oncol. 2020;38(28):3252. [Crossref]  [PubMed]
  10. Comenzo RL, Reece D, Palladini G, Seldin D, Sanchorawala V, Landau H, et al. Consensus guidelines for the conduct and reporting of clinical trials in systemic light-chain amyloidosis. Leukemia. 2012 Nov;26(11):2317-25. [Crossref]  [PubMed]
  11. Sanchorawala V, Finn KT, Fennessey S, Shelton A, Doros G, Zeldis JB, et al. Durable hematologic complete responses can be achieved with lenalidomide in AL amyloidosis. Blood. 2010 Sep 16;116(11):1990-1. [Crossref]  [PubMed]
  12. Dispenzieri A, Buadi F, Laumann K, LaPlant B, Hayman SR, Kumar SK, et al. Activity of pomalidomide in patients with immunoglobulin light-chain amyloidosis. Blood. 2012 Jun 7;119(23):5397-404. [Crossref]  [PubMed]  [PMC]
  13. Palladini G, Merlini G. How I treat AL amyloidosis. Blood. 2022 May 12;139(19):2918-2930. [Crossref]  [PubMed]