Abstract
Immunoglobulin amyloid light-chain (AL) amyloidosis is the most common form of systemic amyloidosis, where the culprit amyloidogenic protein is immunoglobulin light chains produced by marrow clonal plasma cells. AL amyloidosis is an infrequent disease, and since presentation is variable and often nonspecific, diagnosis is often delayed. This results in cumulative organ damage and has a negative prognostic effect. AL amyloidosis can also be challenging on the diagnostic level, especially when demonstration of Congo red-positive tissue is not readily obtained. Since as many as 31 known amyloidogenic proteins have been identified to date, determination of the amyloid type is required. While several typing methods are available, mass spectrometry has become the gold standard for amyloid typing. Upon confirming the diagnosis of amyloidosis, a pursuit for organ involvement is essential, with a focus on heart involvement, even in the absence of suggestive symptoms for involvement, as this has both prognostic and treatment implications. Details regarding initial treatment options, including stem cell transplantation, are provided in this review. AL amyloidosis management requires a multidisciplinary approach with careful patient monito...Continue Reading
Citations
Mar 9, 2017·Annals of Medicine·Eli MuchtarMorie A Gertz
Apr 25, 2017·Biology of Blood and Marrow Transplantation : Journal of the American Society for Blood and Marrow Transplantation·Luis Gerardo Rodríguez-LobatoJoan Bladé
Jan 25, 2017·Current Problems in Cancer·Anit K MankadKeyur B Shah
Jan 21, 2017·Orphanet Journal of Rare Diseases·Martha BaylissMichelle K White
Jun 16, 2017·IJC Heart & Vasculature·Benjamin Thomas FitzgeraldGregory Malcolm Scalia
Oct 21, 2017·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Luis Gerardo Rodríguez-LobatoJoan Bladé
Sep 10, 2017·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·T Liewluck, M Milone
Feb 6, 2018·F1000Research·Jonas Paludo, Stephen M Ansell
Jan 1, 2017·Journal of Patient-reported Outcomes·Michelle K WhiteKristen L McCausland
Apr 5, 2017·Circulation·Mathew S MaurerClaudio Rapezzi
Dec 5, 2018·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Karlijn H G RuttenMonique C Minnema
Oct 4, 2017·Current Medicinal Chemistry·Marta SpodziejaAneta Szymanska
Jul 13, 2019·British Journal of Haematology·Eli MuchtarAngela Dispenzieri
Jul 22, 2018·Neurology·Jennifer KollmerUte Hegenbart
Jun 22, 2020·European Journal of Haematology·Amanda SchulmanVaishali Sanchorawala
Sep 16, 2020·Journal of Internal Medicine·E MuchtarM A Gertz
Nov 11, 2016·International Journal of Hematology·Nagaaki KatohShu-Ichi Ikeda
May 31, 2020·Journal of Nephrology·Roberta FenoglioDario Roccatello
Aug 26, 2017·Current Cardiology Reports·Anit K Mankad, Keyur B Shah
May 7, 2020·Frontiers in Neuroscience·Maja MalmbergKariem Ezzat
Aug 25, 2020·Journal of Clinical Neuromuscular Disease·Sara HuserGeorgios Manousakis
Feb 27, 2021·Journal of Cardiovascular Medicine·Concetta Di NoraUgolino Livi
May 7, 2020·IJC Heart & Vasculature·Maria PapathanasiouPeter Luedike
Dec 23, 2019·JACC. Cardiovascular Imaging·Michele BoldriniMarianna Fontana
Sep 15, 2021·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Alfonso Rivera DuarteVishal Kukreti
May 15, 2020·Acta Haematologica·Osnat Itzhaki Ben Zadok, Ran Kornowski
Dec 2, 2020·Cardiovascular & Hematological Disorders Drug Targets·Azka LatifPreethi William