학술논문
Outcomes of Modified Mayo Stage IIIa and IIIb Cardiac Light-Chain Amyloidosis: Real-World Experience in Clinical Characteristics and Treatment-67 Patients Multicenter Analysis.
Document Type
Academic Journal
Author
Charliński G; Department of Nephrology, Hypertension and Internal Medicine, University of Warmia and Mazury in Olsztyn, 10-719 Olsztyn, Poland.; Department of Hematology and Bone Marrow Transplantation, Nicolaus Copernicus Hospital, 87-100 Torun, Poland.; Steinhardt M; Department of Internal Medicine II, University Hospital of Wurzburg, 97080 Wurzburg, Germany.; Interdisciplinary Amyloidosis Center of Northern Bavaria, University Hospital Wurzburg, 97080 Wurzburg, Germany.; Rasche L; Department of Internal Medicine II, University Hospital of Wurzburg, 97080 Wurzburg, Germany.; Gonzalez-Calle V; a Servicio de Hematología, Hospital Universitario de Salamanca, Instituto de Investigación Biomédica de Salamanca (IBSAL), Centro de Investigación del Cáncer de Salamanca, 37007 Salamanca, Spain.; Peña C; Department of Hematology, Hospital del Salvador, Santiago 7500922, Chile.; Parmar H; Division of Multiple Myeloma, John Theurer Cancer Center at Hackensack, Meridian School of Medicine, Hackensack, NJ 07701, USA.; Wiśniewska-Piąty K; Department of Hematology and Bone Marrow Transplantation, Silesian Medical University, 40-055 Katowice, Poland.; Dávila Valls J; Servicio de Hematologia, Hospital Nuestra Señora de Sonsoles, 05004 Ávila, Spain.; Olszewska-Szopa M; Department of Hematology, Blood Neoplasms and Bone Marrow Transplantation, Wroclaw Medical University, 51-141 Wroclaw, Poland.; Usnarska-Zubkiewicz L; Department of Hematology, Blood Neoplasms and Bone Marrow Transplantation, Wroclaw Medical University, 51-141 Wroclaw, Poland.; Gozzetti A; Hematology, Department of Medical Science, Surgery and Neuroscience, University of Siena, 53100 Siena, Italy.; Ciofini S; Hematology, Department of Medical Science, Surgery and Neuroscience, University of Siena, 53100 Siena, Italy.; Gentile M; Hematology Unit, Department of Onco-Hematology, A.O. of Cosenza, 87100 Cosenza, Italy.; Department of Pharmacy, Health and Nutritional Science, University of Calabria, 87036 Rende, Italy.; Zamagni E; Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia 'Seràgnoli', Università di Bologna, 40126 Bologna, Italy.; Kurlapski M; Department of Hematology and Transplantology, Medical University of Gdańsk, 81-519 Gdańsk, Poland.; Legieć W; Department of Hematology and Bone Marrow Transplantation, St. John of Dukla Oncology Center of Lublin Land, 20-090 Lublin, Poland.; Vesole DH; Division of Multiple Myeloma, John Theurer Cancer Center at Hackensack, Meridian School of Medicine, Hackensack, NJ 07701, USA.; Division of Hematology/Oncology, Medstar Georgetown University Hospital, Washington, DC 20007, USA.; Jurczyszyn A; Plasma Cell Dyscrasias Center, Department of Hematology, Faculty of Medicine, Jagiellonian University College of Medicine, 31-066 Kraków, Poland.
Source
Publisher: MDPI Country of Publication: Switzerland NLM ID: 101526829 Publication Model: Electronic Cited Medium: Print ISSN: 2072-6694 (Print) Linking ISSN: 20726694 NLM ISO Abbreviation: Cancers (Basel) Subsets: PubMed not MEDLINE
Subject
Language
English
ISSN
2072-6694
Abstract
Light-chain amyloidosis (AL) is a rare multisystem disorder characterized by the deposition of misfolded amyloid fibrils derived from monoclonal immunoglobulin light chains in various organs. One of the most common organs involved in AL is the heart, with 50-70% of patients clinically symptomatic at diagnosis. We conducted a multi-center, retrospective analysis of 67 patients diagnosed between July 2012 and August 2022 with the European 2012 modification of Mayo 2004 stage III cardiac AL. The most important factors identified in the univariate Cox analysis contributing to a longer OS included Eastern Cooperative Oncology Group performance status (ECOG PS) ≤ 1, New York Heart Association functional classification (NYHA FC) ≤ 2, the use of autologous stem cell transplantation (ASCT) after induction treatment, achieving a hematological response (≥very good partial response) and cardiac (≥partial response) response after first-line treatment. The most important prognostic factors with the most significant impact on OS improvement in patients with modified Mayo stage III cardiac AL identified by multivariate Cox analysis are ECOG PS ≤ 1, NYHA FC ≤ 2, and achieving hematological response ≥ VGPR and cardiac response ≥ PR after first-line treatment.