TY - CHAP
T1 - Immunoglobulin light chain systemic amyloidosis
AU - Dispenzieri, Angela
AU - Merlini, Giampaolo
N1 - Publisher Copyright:
© Springer International Publishing Switzerland 2016.
PY - 2016
Y1 - 2016
N2 - Immunoglobulin light chain amyloidosis (AL) is a rare, complex disease caused by misfolded free light chains produced by a usually small, indolent plasma cell clone. Effective treatments exist that can alter the natural history, provided that they are started before irreversible organ damage has occurred. The cornerstones of the management of AL amyloidosis are early diagnosis, accurate typing, appropriate risk-adapted therapy, tight follow-up, and effective supportive treatment. The suppression of the amyloidogenic light chains using the cardiac biomarkers as guide to choose chemotherapy is still the mainstay of therapy. There are exciting possibilities ahead, including the study of oral proteasome inhibitors, antibodies directed at plasma cell clone, and finally antibodies attacking the amyloid deposits are entering the clinic, offering unprecedented opportunities for radically improving the care of this disease.
AB - Immunoglobulin light chain amyloidosis (AL) is a rare, complex disease caused by misfolded free light chains produced by a usually small, indolent plasma cell clone. Effective treatments exist that can alter the natural history, provided that they are started before irreversible organ damage has occurred. The cornerstones of the management of AL amyloidosis are early diagnosis, accurate typing, appropriate risk-adapted therapy, tight follow-up, and effective supportive treatment. The suppression of the amyloidogenic light chains using the cardiac biomarkers as guide to choose chemotherapy is still the mainstay of therapy. There are exciting possibilities ahead, including the study of oral proteasome inhibitors, antibodies directed at plasma cell clone, and finally antibodies attacking the amyloid deposits are entering the clinic, offering unprecedented opportunities for radically improving the care of this disease.
KW - Biomarkers
KW - Cardiac amyloidosis
KW - Chemotherapy
KW - Immunoglobulin light chain amyloidosis
KW - Immunotherapy
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U2 - 10.1007/978-3-319-40320-5_15
DO - 10.1007/978-3-319-40320-5_15
M3 - Chapter
C2 - 27696268
AN - SCOPUS:84989855736
T3 - Cancer Treatment and Research
SP - 273
EP - 318
BT - Cancer Treatment and Research
PB - Kluwer Academic Publishers
ER -