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Updated: Dec 25, 2025

Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
Supportive Care in AL Amyloidosis
M Teresa Cibeira1, José T Ortiz-Pérez2, Luis F Quintana3
1Hematology Department, Amyloidosis and Myeloma Unit, Hospital Clínic of Barcelona, University of Barcelona, IDIBAPS, Barcelona, Spain, mcibeira@clinic.cat.
Abstract:
Immunoglobulin light-chain (AL) amyloidosis is a systemic disease characterized by the production and deposition of light chain-derived amyloid fibrils in different organs. Prompt treatment directed to the underlying plasma cell clone is crucial in order to achieve a rapid, deep and durable hematologic response. The decrease in the production of the amyloidogenic light chains is a required condition to obtain the organ response, which is commonly delayed. Meanwhile, supportive treatment is aimed to maintain quality of life of these patients and preserve their involved organs' function. From simple measures, such as salt restriction or compressive stockings, to very complex interventions, such as heart transplantation in very selected patients with isolated severe cardiac involvement, this supportive care is essential and has to be necessarily included in the multidisciplinary management of this disease.
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