Figure 4.
Common pitfalls in the management of patients with AL amyloidosis. A presymptomatic biomarker-based diagnosis is possible in patients at risk (subjects with MGUS and abnormal FLC ratio). Treating patients at early stages facilitates the access to effective therapies and can improve survival. The diagnostic pathways for AL and non-AL amyloidosis are different and the choice depends on the presence or absence of a monoclonal component. AL amyloidosis progresses more rapidly, but available treatments can rapidly reverse the course of the disease. The diagnosis of AL amyloidosis should not be delayed. Positive cardiac scintigraphy with bone tracers is not enough to establish a diagnosis of ATTR amyloidosis in a patient with a monoclonal component. Uncharacterized amyloid deposits on a tissue biopsy in a patient with a monoclonal component are not enough to establish a diagnosis of AL amyloidosis. Prespecified treatment duration and/or number of cycles should be avoided in AL amyloidosis. The goal is rapid and deep HR and if it is not reached, rescue therapy is needed. Organ response can sometimes be delayed.