Summary
Immunoglobulin light chain amyloidosis is a clonal plasma cell dyscrasia, historically associated with a very poor prognosis. Prompt diagnosis is critical to preserve organ function and improve survival in immunoglobulin light chain amyloidosis patients. The severity of cardiac involvement and response to treatment are the most important prognostic factors. Serum free light chain ratio and cardiac biomarkers troponin T and N-terminal pro-brain natriuretic peptide are powerful tools for the evaluation of prognosis and treatment response. Historically, treatment with autologous stem cell transplantation appears to offer a survival benefit, but is only an option in a minority of patients. IMiDs, and especially proteasome inhibitors, have shown promising activity in immunoglobulin light chain amyloidosis. Supportive care should be integrated in the treatment plan and requires a multidisciplinary approach. These guidelines summarise a consensus of the myeloma subcommittee of the Belgian Hematological Society on diagnosis, cytoreductive and supportive treatment of immunoglobulin light chain amyloidosis, based on an extended review of the literature. Where applicable, comments were added with respect to the Belgian reimbursement modalities.
(BELG J HEMATOL 2015;6(5):187–94)
