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Clinical features depend on organs involved but can include heart failure with preserved ejection fraction, nephrotic syndrome, hepatic dysfunction, peripheral\/autonomic neuropathy, and \u201catypical smoldering multiple myeloma or monoclonal gammopathy undetermined significance (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 for diagnosis. Invasive organ biopsy is not required in 85% of patients. Verification that amyloid is composed of immunoglobulin light chains is mandatory. The gold standard is laser capture mass spectroscopy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Prognosis<\/jats:title>\n                    <jats:p>N\u2010terminal pro\u2010brain natriuretic peptide (NT\u2010proBNP), serum troponin T, and difference between involved and uninvolved immunoglobulin free light chain (FLC) values are used to classify patients into four groups of similar size; median survivals are 94.1, 40.3, 14.0, and 5.8 months.<\/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. Stem cell transplant (SCT) is preferred, but only 20% of patients are eligible. Requirements for safe SCT include systolic blood pressure &gt;90\u2009mmHg, troponin T\u2009&lt;\u20090.06\u2009ng\/mL and serum creatinine \u22641.7 mg\/dL. Nontransplant candidates can be offered cyclophosphamide\u2010bortezomib\u2010dexamethasone or daratumumab\u2010containing regimens as it appears to be highly active in AL amyloidosis.<\/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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ajh.25819","type":"journal-article","created":{"date-parts":[[2020,4,8]],"date-time":"2020-04-08T05:29:06Z","timestamp":1586323746000},"page":"848-860","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":132,"title":["Immunoglobulin light chain amyloidosis: 2020 update on diagnosis, prognosis, and treatment"],"prefix":"10.1002","volume":"95","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 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,4,28]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-11-125526"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019002762"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-009-8163-9"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2013.50.0892"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-017-15424-3"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.5858\/134.4.545"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2458-12-974"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.12286"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/gfs492"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mayocp.2018.08.041"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamacardio.2018.2093"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.3816\/CLML.2010.n.027"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11886-011-0239-5"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.acvd.2008.11.006"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrrheum.2010.84"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.critrevonc.2010.05.001"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pmrj.2008.11.011"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcu.20552"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11886-010-0108-7"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2006.07.107"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-010-1028-8"},{"key":"e_1_2_7_23_1","first-page":"105","article-title":"Screening panels for monoclonal gammopathies: time to change","volume":"30","author":"Katzmann JA","year":"2009","journal-title":"Clin Biochem Rev"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cca.2009.04.024"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2008.307"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1373\/clinchem.2008.117143"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3181abdfa7"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-010-0909-5"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-009-0857-9"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.20101"},{"key":"e_1_2_7_31_1","unstructured":"TraftonW LibbeyC.Abdominal fat tissue aspirate procedure[Internet]. 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