GERAMY - Network for Systemic Amyloid light-Chain (AL) Amyloidosis

Systemic amyloid light-chain (AL) amyloidosis is a rare but fatal protein folding disorder. It is caused by monoclonal plasma cells in the bone marrow producing amyloidogenic light chain proteins, which accumulate in several organs. Early diagnosis is very difficult because initial symptoms are unspecific. Cause of death is usually heart failure due to excessive cardiac amyloid deposition. To date, the only evidence-based-therapy to stop the production of amyloid precursors is cytoreductive treatment of the underlying B cell clone, which is often very toxic in this group of fragile patients.

In this consortium, a controlled clinical trial will be performed to test the efficacy and safety of epigallocatechin-3-gallate (EGCG) in patients with cardiac AL amyloidosis. In parallel, the molecular basis of the disease will be investigated using genetic, biochemical, cell biological and high-resolution biophysical methods to reveal new molecular targets and to further refine diagnosis of disease. This translational, interdisciplinary approach will increase our knowledge of the pathogenesis of AL amyloidosis and enable new developments to improve diagnosis, therapy and prognosis of affected patients.