Amyloidosis (Amyloid light-chain (AL)) (short version)

Amyloidoses are rare protein folding diseases in which proteins are deposited as insoluble fibrillar aggregates as a result of a conformational change. This can be systemic (site of production and deposition different) or localized (site of production and deposition identical). Nomenclature is based on the amyloidogenic protein, which has superseded the previous classification into primary and secondary amyoidosis. Systemic amyloidoses are potentially life-threatening complications of monoclonal gammopathies (light chains, AL amyloidoses), age-related diseases (transthyretin, nonmutated; ATTRwt), chronic inflammation (serum amyloid A, AA amyloidoses), or they run in families as part of a monogenetic disease (most commonly transthyretin, TTR amyloidoses). The causal treatment of AL amyloidosis is the reduction of amyloid-forming light chains by immunochemotherapy. Early diagnosis of the disease is essential. At this stage, patients quality for immuno-chemotherapy and can therefore still be treated effectively, thus avoiding further functional deterioration of the organs.

Patients should be presented to an amyloidosis center before initiating therapy, if possible. A list of centers can be found on the homepage of the German Society for Amyloid Diseases (http://www.amyloid.de/). There are also centers in Switzerland and Austria (Swiss Amyloidosis Network of the University Hospital Zurich https://www.usz.ch/krankheit/amyloidose/; Interdisciplinary Amyloidosis Center of the Medical University of Vienna).

The current treatment algorithm is depicted in Figure 1 and Figure 2.

Conflicts of interest can be found in the full German version of the guideline.