{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T23:37:34Z","timestamp":1769125054075,"version":"3.49.0"},"reference-count":20,"publisher":"Walter de Gruyter GmbH","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2016,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The detection and quantification of amyloidogenic monoclonal light chains are necessary for the diagnosis and evaluation of response to treatment in AL amyloidosis. However, the amyloid clone is often small and difficult to detect. We report the case of a 68-year-old man who was referred to our Center in April 2013 after syncope and the identification of left ventricular hypertrophy at echocardiography, suspected for amyloidosis. A commercial agarose gel electrophoresis immunofixation (IFE) did not reveal monoclonal components in serum and urine. The \u03ba serum free light chain (FLC) concentration was 21.5 mg\/L, \u03bb 33 mg\/L (\u03ba\/\u03bb ratio 0.65), NT-proBNP 9074 ng\/L (u.r.l. &lt;332 ng\/L) and an echocardiogram confirmed characteristic features of amyloidosis. The abdominal fat aspiration was positive and the amyloid typing by immune-electron microscopy revealed \u03bb light chains deposits. A high-resolution (hr) IFE of serum and urine showed a faint monoclonal \u03bb component in the urine. A bone marrow biopsy showed 8% plasma cells (BMPC) and a kappa\/lambda light-chain restriction with \u03bb light chain on immunofluorescence. The diagnosis of AL (\u03bb) amyloidosis with cardiac involvement was made. In May 2013, patient was started on cyclophosphamide, bortezomib and dexamethasone. After six cycles, serum and urine hr-IFE were negative, the bone marrow biopsy showed 3% BMPC without light chain restriction by immunofluorescence, and a decrease of NT-proBNP was observed (5802 ng\/L).Thus, treatment was discontinued. In this patient the amyloid clone could be detected only by in house hr-IFE of urine and bone marrow examination. The detection of the small dangerous amyloidogenic clone should be pursued with a combination of high-sensitivity techniques, including assessment of BMPC clonality. Studies of novel tools, such as mass spectrometry on serum and next-generation flow cytometry analysis of the bone marrow, for detecting plasma cell clones in AL amyloidosis and other monoclonal light chain-related disorders are warranted.<\/jats:p>","DOI":"10.1515\/cclm-2015-0847","type":"journal-article","created":{"date-parts":[[2015,12,17]],"date-time":"2015-12-17T17:18:27Z","timestamp":1450372707000},"source":"Crossref","is-referenced-by-count":8,"title":["A patient with AL amyloidosis with negative free light chain results"],"prefix":"10.1515","volume":"54","author":[{"given":"Paolo","family":"Milani","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Veronica","family":"Valentini","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanni","family":"Ferraro","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marco","family":"Basset","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesca","family":"Russo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"Foli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanni","family":"Palladini","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giampaolo","family":"Merlini","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"374","reference":[{"key":"ref91","doi-asserted-by":"crossref","first-page":"4541","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","year":"2012","journal-title":"J Clin Oncol"},{"key":"ref151","doi-asserted-by":"crossref","first-page":"4957","DOI":"10.1182\/blood-2009-07-230722","article-title":"Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens","volume":"114","year":"2009","journal-title":"Blood"},{"key":"ref161","first-page":"1025","article-title":"The Pavia approach to clinical protein analysis","volume":"39","year":"2001","journal-title":"Clin Chem Lab Med"},{"key":"ref121","doi-asserted-by":"crossref","first-page":"1517","DOI":"10.1373\/clinchem.2009.126664","article-title":"Screening panels for detection of monoclonal gammopathies","volume":"55","year":"2009","journal-title":"Clin Chem"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1373\/clinchem.2008.117143","article-title":"Identification of amyloidogenic light chains requires the combination of serum-free light chain assay with immunofixation of serum and urine","volume":"55","year":"2009","journal-title":"Clin Chem"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"2239","DOI":"10.1182\/blood-2014-11-609883","article-title":"A practical approach to the diagnosis of systemic amyloidoses","volume":"125","year":"2015","journal-title":"Blood"},{"key":"ref101","doi-asserted-by":"crossref","first-page":"2520","DOI":"10.1182\/blood-2006-03-001164","article-title":"Dangerous small B-cell clones","volume":"108","year":"2006","journal-title":"Blood"},{"key":"ref171","doi-asserted-by":"crossref","first-page":"5124","DOI":"10.1182\/blood-2013-01-453001","article-title":"Systemic light chain amyloidosis: an update for treating physicians","volume":"121","year":"2013","journal-title":"Blood"},{"key":"ref71","doi-asserted-by":"crossref","first-page":"5124","DOI":"10.1182\/blood-2013-01-453001","article-title":"Systemic light chain amyloidosis: an update for treating physicians","volume":"121","year":"2013","journal-title":"Blood"},{"key":"ref191","doi-asserted-by":"crossref","first-page":"4541","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","year":"2012","journal-title":"J Clin Oncol"},{"key":"ref51","doi-asserted-by":"crossref","first-page":"4957","DOI":"10.1182\/blood-2009-07-230722","article-title":"Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens","volume":"114","year":"2009","journal-title":"Blood"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"1517","DOI":"10.1373\/clinchem.2009.126664","article-title":"Screening panels for detection of monoclonal gammopathies","volume":"55","year":"2009","journal-title":"Clin Chem"},{"key":"ref131","doi-asserted-by":"crossref","first-page":"2239","DOI":"10.1182\/blood-2014-11-609883","article-title":"A practical approach to the diagnosis of systemic amyloidoses","volume":"125","year":"2015","journal-title":"Blood"},{"key":"ref141","doi-asserted-by":"crossref","first-page":"1844","DOI":"10.1182\/blood-2011-07-365510","article-title":"Reliable typing of systemic amyloidoses through proteomic analysis of subcutaneous adipose tissue","volume":"119","year":"2012","journal-title":"Blood"},{"key":"ref111","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1373\/clinchem.2008.117143","article-title":"Identification of amyloidogenic light chains requires the combination of serum-free light chain assay with immunofixation of serum and urine","volume":"55","year":"2009","journal-title":"Clin Chem"},{"key":"ref61","first-page":"1025","article-title":"The Pavia approach to clinical protein analysis","volume":"39","year":"2001","journal-title":"Clin Chem Lab Med"},{"key":"ref81","first-page":"218","article-title":"The degrees of plasma cell clonality and marrow infiltration adversely influence the prognosis of AL amyloidosis patients","volume":"84","year":"1999","journal-title":"Haematologica"},{"key":"ref01","doi-asserted-by":"crossref","first-page":"2520","DOI":"10.1182\/blood-2006-03-001164","article-title":"Dangerous small B-cell clones","volume":"108","year":"2006","journal-title":"Blood"},{"key":"ref181","first-page":"218","article-title":"The degrees of plasma cell clonality and marrow infiltration adversely influence the prognosis of AL amyloidosis patients","volume":"84","year":"1999","journal-title":"Haematologica"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"1844","DOI":"10.1182\/blood-2011-07-365510","article-title":"Reliable typing of systemic amyloidoses through proteomic analysis of subcutaneous adipose tissue","volume":"119","year":"2012","journal-title":"Blood"}],"container-title":["Clinical Chemistry and Laboratory Medicine (CCLM)"],"original-title":[],"link":[{"URL":"https:\/\/www.degruyter.com\/document\/doi\/10.1515\/cclm-2015-0847\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,4,21]],"date-time":"2021-04-21T23:50:59Z","timestamp":1619049059000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.degruyter.com\/document\/doi\/10.1515\/cclm-2015-0847\/html"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,1,1]]},"references-count":20,"journal-issue":{"issue":"6"},"URL":"https:\/\/doi.org\/10.1515\/cclm-2015-0847","relation":{},"ISSN":["1437-4331","1434-6621"],"issn-type":[{"value":"1437-4331","type":"electronic"},{"value":"1434-6621","type":"print"}],"subject":[],"published":{"date-parts":[[2016,1,1]]}}}