English 
Italian RASSEGNE - Reviews
Volume:
Biochimica Clinica 2013; 37(5) 347-351
Published on-line:
DOI:
Le catene leggere libere circolanti nella gestione del paziente con amiloidosi AL
AUTHORS
1Centro per lo Studio e la Cura delle Amiloidosi Sistemiche e Laboratori di Biotecnologie, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo e Dipartimento di Medicina Molecolare, Università di Pavia
2Laboratorio di Analisi Chimico Cliniche, Fondazione IRCCS Policlinico San Matteo, Pavia
ABSTRACT
Free light chain (FLC) assays in the management of patients with AL amyloidosis
In light chain (AL) amyloidosis monoclonal FLC are not only a marker of the disease, but the toxic agent responsible for the organ damage. The possibility of measuring FLC provides an extraordinary means for managing AL amyloidosis. The quantitation of FLC, together with immunofixation of both serum and urine, allows optimal (98%-100%) sensitivity in detecting the amyloidogenic monoclonal protein. In combination with cardiac troponins and amino-terminal pronatriuretic type B peptide, FLC measurement grants an accurate prognostic stratification, which is important both in tailoring the therapeutic approach in individual patients and in designing and interpreting clinical trials. Chemotherapy targeting the amyloidogenic plasma cell clone succeeds in restoring organ function and prolonging survival only if it obtains a marked and persistent reduction of FLC, so much so that current response criteria are based on FLC measurement.