Abstract

BRULC, Erika B. et al. Recommendations for the treatment of AL amyloidosis. Medicina (B. Aires) [online]. 2022, vol.82, n.4, pp.591-604. ISSN 0025-7680.

Introduction: Immunoglobulin light chain (AL) amyloidosis is a rare disease. Treatment is chal lenging, justified in part by systemic compromise and limited scientific evidence. Objectives: Develop evidence-based recommendations that allow adequate treatment of patients with amyloidosis AL. Methods: A list of PICO format questions focused on the effectiveness and safety of amyloidosis AL treatment was generated. PubMed, Cochrane and Epistemonikos were searched. The levels of evidence and grades of recommendation were based on the GRADE system. Results: 11 recommendations were generated. In selected patients with amyloidosis AL, autologous hematopoietic stem cell transplantation (ASCT) is recommended after induction with bortezomib-based regimens and conditioning with melphalan, since it could deepen the hematological and organ response, its durability and improve survival. In patients not eligible for ASCT, first-line treatment with bortezomib-based regimens is recommended, since it is likely to achieve a higher rate of hematological and organ response and improve survival. In patients with a contraindication or inaccessibility to bortezomib, treatment with alkylating agents and corticosteroids is recommended, since they are likely to achieve haematological and organ response and improve survival. Discussion: These treatment recommendations are based on the available evidence and the experience of the panel of experts, in a scenario of limited available resources, according to developing countries.

Keywords : Amyloidosis; Immunoglobulin light chain amyloidosis; Clinical practice guidelines; Recommendations; GRADE approac.

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