{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,21]],"date-time":"2026-07-21T07:27:15Z","timestamp":1784618835555,"version":"3.55.0"},"reference-count":26,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,10,6]],"date-time":"2022-10-06T00:00:00Z","timestamp":1665014400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:p>Cardiac amyloidosis (CA) represents a myocardial disorder developed by fibril deposition of a heterogeneous group of misfolding proteins. Despite being rare, a high clinical index of suspicion and novel advanced diagnostic methods seem to facilitate its early recognition. Currently nine types of cardiac amyloidosis have been described with AL and ATTR being the most common. Light chain amyloidosis (AL) is a life-threatening disease, resulting from clonal plasma cells that produce amyloidogenic light chain fragments causing organ damage including the heart. Morbidity and mortality of these patients is strongly associated with the severity of cardiac involvement. Thus, early and precise diagnosis is crucial for prompt treatment initiation. In this study, we retrospectively analyzed data of 36 consecutive patients who were diagnosed with AL amyloidosis and treated in our center over the past 15 years. Heart involvement was present in 33 (92%) of them while 76% had severe cardiac disease as of stage IIIa and IIIb, according to the Mayo2004\/European staging system. Almost one third of these patients experienced an early death occurring the first five months of diagnosis. To capture everyday clinical practice, we provide details on clinical presentation, diagnostic challenges, and outcome of these patients.<\/jats:p>","DOI":"10.3389\/fonc.2022.944503","type":"journal-article","created":{"date-parts":[[2022,10,6]],"date-time":"2022-10-06T13:29:41Z","timestamp":1665062981000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":7,"title":["Real world data on light chain cardiac amyloidosis: Still a delayed diagnosis"],"prefix":"10.3389","volume":"12","author":[{"given":"Sofia","family":"Chatzileontiadou","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Zegkos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina","family":"Frouzaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Athanasia","family":"Apsemidou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georgios","family":"Efthimiadis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Despoina","family":"Parcharidou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Papaioannou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,10,6]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2020.100720","article-title":"The diagnostic challenges of cardiac amyloidosis: A practical approach to the two main types","volume":"45","author":"Varga","year":"2021","journal-title":"Blood Rev"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1080\/13506129.2018.1549825","article-title":"Amyloid nomenclature 2018: recommendations by the international society of amyloidosis (ISA) nomenclature committee","volume":"25","author":"Benson","year":"2018","journal-title":"Amyloid"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehab072","article-title":"Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESC working group on myocardial and pericardial diseases","volume":"42","author":"Garcia-Pavia","year":"2021","journal-title":"Eur Heart J"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23333","article-title":"Clinical features and treatment outcome of primary systemic light-chain amyloidosis in Korea: Results of multicenter analysis","volume":"88","author":"Jun","year":"2013","journal-title":"Am J Hematol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1007\/s11899-021-00626-4","article-title":"Amyloidosis with cardiac involvement: Identification, characterization, and management","volume":"16","author":"Jamal","year":"2021","journal-title":"Curr Hematol Malig Rep"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-10-875252","article-title":"Staging systems use for risk stratification of systemic amyloidosis in the era of high-sensitivity troponin T assay","volume":"133","author":"Muchtar","year":"2019","journal-title":"Blood"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1002\/clc.23572","article-title":"Cardiac amyloidosis-a review of current literature for the practicing physician","volume":"44","author":"Ash","year":"2021","journal-title":"Clin Cardiol"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2016-310704","article-title":"Light-chain cardiac amyloidosis: strategies to promote early diagnosis and cardiac response","volume":"103","author":"Grogan","year":"2017","journal-title":"Heart"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1182\/hematology.2020006913","article-title":"Management of AL amyloidosis in 2020","volume":"2020","author":"Palladini","year":"2020","journal-title":"Hematol Am Soc Hematol Educ Program."},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2011.37.7614","article-title":"New criteria for response to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on survival outcomes","volume":"30","author":"Palladini","year":"2012","journal-title":"J Clin Oncol"},{"key":"B11","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1038\/s41408-020-0306-5","article-title":"A validated composite organ and hematologic response model for early assessment of treatment outcomes in light chain amyloidosis","volume":"10","author":"Sidana","year":"2020","journal-title":"Blood Cancer J"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-12-473066","article-title":"A European collaborative study of treatment outcomes in 346 patients with cardiac stage III AL amyloidosis","volume":"121","author":"Wechalekar","year":"2013","journal-title":"Blood"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2004.03.029","article-title":"Serum cardiac troponins and n-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis","volume":"22","author":"Dispenzieri","year":"2004","journal-title":"J Clin Oncol"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2019.218859","article-title":"Predicting survival in light chain amyloidosis","volume":"104","author":"Palladini","year":"2019","journal-title":"Haematologica"},{"key":"B15","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1159\/000495455","article-title":"Recent advances in the diagnosis, risk stratification, and management of systemic light-chain amyloidosis","volume":"141","author":"Vaxman","year":"2019","journal-title":"Acta Haematol"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1007\/s40271-017-0273-5","article-title":"Light chain (AL) amyloidosis: The journey to diagnosis","volume":"11","author":"McCausland","year":"2018","journal-title":"PatientFigure 1. Kaplan-Meier curve of overall survival (OS) for 36 patients with AL amyloidosis seen at the 1st Internal Medicine Department, AHEPA University Hospital, Thessaloniki, Greece. Median OS was 14 months."},{"key":"B17","doi-asserted-by":"publisher","first-page":"90","DOI":"10.1038\/s41408-021-00483-7","article-title":"Immunoglobulin light chain amyloidosis diagnosis and treatment algorithm 2021","volume":"11","author":"Hasib Sidiqi","year":"2021","journal-title":"Blood Cancer J"},{"key":"B18","doi-asserted-by":"publisher","first-page":"389","DOI":"10.1016\/j.ccl.2021.04.010","article-title":"Advances in diagnosis and treatment of cardiac and renal amyloidosis","volume":"39","author":"Law","year":"2021","journal-title":"Cardiol Clin"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.6004\/jadpro.2019.10.8.4","article-title":"AL amyloidosis: Unfolding a complex disease","volume":"10","author":"Lu","year":"2019","journal-title":"J Adv Pract Oncol"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1002\/cam4.3683","article-title":"AL amyloidosis: The effect of fluorescent in situ hybridization abnormalities on organ involvement and survival","volume":"10","author":"Ozga","year":"2020","journal-title":"Cancer Med"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1159\/000506617","article-title":"When to suspect a diagnosis of amyloidosis","volume":"143","author":"Vaxman","year":"2020","journal-title":"Acta Haematol"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1159\/000506760","article-title":"Supportive care in AL amyloidosis","volume":"143","author":"Cibeira","year":"2020","journal-title":"AHA"},{"key":"B23","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1056\/NEJMoa2028631","article-title":"Daratumumab-based treatment for immunoglobulin light-chain amyloidosis","volume":"385","author":"Kastritis","year":"2021","journal-title":"N Engl J Med"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1007\/s40265-022-01705-3","article-title":"Daratumumab: A review in newly diagnosed systemic light chain amyloidosis","volume":"82","author":"Blair","year":"2022","journal-title":"Drugs"},{"key":"B25","doi-asserted-by":"publisher","first-page":"8","DOI":"10.1161\/CIRCULATIONAHA.121.055953","article-title":"Doxycycline combined with bortezomib-Cyclophosphamide-Dexamethasone chemotherapy for newly diagnosed cardiac light-chain amyloidosis: A multicenter randomized controlled trial","volume":"145","author":"Shen","year":"2022","journal-title":"Circulation"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.26569","article-title":"Immunoglobulin light chain amyloidosis: 2022 update on diagnosis, prognosis, and treatment","volume":"97","author":"Gertz","year":"2022","journal-title":"Am J Hematol"}],"container-title":["Frontiers in Oncology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2022.944503\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,10,6]],"date-time":"2022-10-06T13:29:43Z","timestamp":1665062983000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2022.944503\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,10,6]]},"references-count":26,"alternative-id":["10.3389\/fonc.2022.944503"],"URL":"https:\/\/doi.org\/10.3389\/fonc.2022.944503","relation":{},"ISSN":["2234-943X"],"issn-type":[{"value":"2234-943X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,10,6]]},"article-number":"944503"}}