[Amyloid Registry Kiel : An interim balance]

Amyloid is a pathologic fibrillar aggregation of polypeptides in a cross-β-sheet conformation. Amyloidoses are caused by the deposition of amyloid and may occur as cerebal and extracerebral diseases. A total of 35 different amyloid proteins have been identified. The Amyloid Registry Kiel was founded in 2009 at the Department of Pathology of the Christian-Albrechts-University Kiel. It currently houses more than 6700 cases of diverse anatomical origin with histologically confirmed amyloid and amyloidosis. This large series of cases provides unique insights into an otherwise rare disease. This includes the validation of novel dyes for the histological recognition and diagnosis of amyloid (luminescent conjugated polythiophenes), the in-depth analysis of amyloid associated with carpal tunnel syndrome, the distribution pattern of diverse amyloids in biopsies of the gastrointestinal tract and the prognostic significance of immunoglobulin light chain-derived AL amyloid load in cardiac biopsies. Finally, the application of MALDI (matrix-assisted laser desorption/ionization) mass spectrometry imaging provided valuable insights into the complexity of amyloid deposits, which consist of a diagnostic disease-specific and amyloid-specific protein/peptide mixture.