Open AccessPrognosis and Staging of AL Amyloidosis
Author(s) -
Tobias Dittrich,
Christoph Kimmich,
Ute Hegenbart,
Stefan Schönland
Publication year - 2020
Publication title -
acta haematologica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.574
H-Index - 56
eISSN - 1421-9662
pISSN - 0001-5792
DOI - 10.1159/000508287
Subject(s) - al amyloidosis , medicine , amyloidosis , context (archaeology) , multiple myeloma , intensive care medicine , fluorescence in situ hybridization , oncology , immunoglobulin light chain , immunology , biology , paleontology , antibody , biochemistry , gene , chromosome
The treatment options for systemic light chain amyloidosis (AL) are currently widening in an unprecedented way, brought about by an expanding arsenal of anti-myeloma therapy as well as by novel approaches to target toxic light chains and, most recently, deposited amyloid directly. In this context, accurate estimates of prognosis in AL, which allow for reliable patient advice and for example comparison of different therapies, are particularly important to clinicians. Some biomarkers and especially the genetic background of the underlying clonal disease as evaluated by interphase fluorescence in situ hybridization even have predictive value, enabling an appropriate treatment selection. Derived from the most frequently involved organs in AL, heart and kidney, this review focuses on overall survival and renal survival. A comprehensive overview and summary of reported prognostic factors and biomarkers in AL is given and the most important and validated factors are highlighted. Finally, established staging systems in AL as well as validated and perspective response criteria are presented.