Therapy and management of systemic AL (primary) amyloidosis.

The optimal treatment of immunoglobulin light chain amyloidosis (AL) patients requires early diagnosis, correct amyloid typing, effective treatment and careful supportive therapy. In the last few years the therapeutic arsenal for the management of AL has been considerably enriched. Cardiac dysfunction can be accurately monitored by measuring the serum concentration of natriuretic peptide type-B and cardiac troponins and the quantitative test for circulating free light chains allows an easy assessment of haematological response to chemotherapy. These new tools can be combined in order to maximise the improvement of organ dysfunction and minimise toxicity, adapting the intervention to each patient.