{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T10:10:05Z","timestamp":1792145405302,"version":"4.4.0"},"reference-count":119,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,2,22]],"date-time":"2026-02-22T00:00:00Z","timestamp":1771718400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,2,22]],"date-time":"2026-02-22T00:00:00Z","timestamp":1771718400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Disease Overview<\/jats:title>\n                    <jats:p>AL amyloidosis is a clonal plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include HFpEF, nephrotic syndrome, hepatic dysfunction, peripheral\/autonomic neuropathy, and \u201catypical smoldering multiple myeloma or MGUS.\u201d<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Diagnosis<\/jats:title>\n                    <jats:p>Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple\u2010green birefringence is required. Organ biopsy is not required in 85% of patients. Verification that amyloid is composed of immunoglobulin light chains is mandatory.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Prognosis<\/jats:title>\n                    <jats:p>N\u2010terminal pro\u2013brain natriuretic peptide (NT\u2010proBNP or BNP), serum troponin T (or I), and difference between involved and uninvolved immunoglobulin free light chain values are used to classify patients into four stages; 5\u2009year survivals are 82%, 62%, 34%, and 20% respectively.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Therapy<\/jats:title>\n                    <jats:p>All patients with a systemic amyloid syndrome require therapy to prevent deposition of amyloid in other organs and prevent progressive organ failure. Current first line therapy with the best outcome is daratumumab, bortezomib, cyclophosphamide, and dexamethasone. The goal of therapy is a\u2009\u2265\u2009VGPR. In patients failing to achieve this depth of response, options for consolidation include pomalidomide, stem cell transplantation, and venetoclax. T\u2010cell redirecting therapies, both bispecific antibodies and car T, show high level activity and may soon become standard second\u2010line therapy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Future Challenges<\/jats:title>\n                    <jats:p>Delayed diagnosis remains a major obstacle to initiating effective therapy prior to the development of end\u2010stage organ failure. An antibody to deplete deposited \u03ba fibrils has reported benefit in patients with cardiomyopathy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ajh.70246","type":"journal-article","created":{"date-parts":[[2026,2,22]],"date-time":"2026-02-22T12:40:01Z","timestamp":1771764001000},"page":"1056-1069","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Immunoglobulin Light Chain Amyloidosis: 2026 Update on Diagnosis, Prognosis, and Treatment"],"prefix":"10.1002","volume":"101","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3853-5196","authenticated-orcid":false,"given":"Morie A.","family":"Gertz","sequence":"first","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota 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