000128019 001__ 128019
000128019 005__ 20240228145530.0
000128019 0247_ $$2doi$$a10.1182/blood-2017-02-767475
000128019 0247_ $$2pmid$$apmid:28550043
000128019 0247_ $$2ISSN$$a0006-4971
000128019 0247_ $$2ISSN$$a1528-0020
000128019 0247_ $$2altmetric$$aaltmetric:20667488
000128019 037__ $$aDKFZ-2017-04041
000128019 041__ $$aeng
000128019 082__ $$a610
000128019 1001_ $$00000-0002-6442-8255$$aDittrich, Tobias$$b0
000128019 245__ $$aAL amyloidosis patients with low amyloidogenic free light chain levels at first diagnosis have an excellent prognosis.
000128019 260__ $$aStanford, Calif.$$bHighWire Press$$c2017
000128019 3367_ $$2DRIVER$$aarticle
000128019 3367_ $$2DataCite$$aOutput Types/Journal article
000128019 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1511351427_28996
000128019 3367_ $$2BibTeX$$aARTICLE
000128019 3367_ $$2ORCID$$aJOURNAL_ARTICLE
000128019 3367_ $$00$$2EndNote$$aJournal Article
000128019 520__ $$aThe difference between involved minus uninvolved serum free light chains (dFLC) has been established as an invaluable hematologic parameter in systemic amyloid light chain (AL) amyloidosis. However, patients with an initial dFLC level <50 mg/L are currently deemed not evaluable for response to therapy. Therefore, we aimed to characterize this subgroup of patients and to define novel hematologic response parameters. We retrospectively analyzed 783 AL patients newly diagnosed at our center between 2002 and 2016. Patients with a dFLC level <50 mg/L showed smaller bone marrow plasmacytosis compared to patients with a dFLC level ≥50 mg/L (7% vs 10%, P < .001), but no significant differences in all analyzed chromosomal aberrations. Cardiac involvement was less frequent (45% vs 80%, P < .001) and less severe (Mayo 2004 stage III: 18% vs 51%, P < .001), whereas kidney involvement was more prevalent (83% vs 53%, P < .001) and proteinuria was higher (7.3 g/L vs 5.0 g/L, P < .001). In multivariate analyses, a dFLC level <50 mg/L appeared to be an independent prognostic factor with respect to overall survival (hazard ratio [HR] = 0.50, P = .003) and renal survival (HR = 0.56, P = .020). Patients with a dFLC level <50 mg/L showed a higher proportion of complete hematologic response after first-line therapy compared to patients with a dFLC level ≥50 mg/L (39% vs 9%, P < .001). Receiver-operating characteristics analysis identified a low-dFLC partial response (dFLC <10 mg/L for patients with a dFLC between 20 and 50 mg/L), which predicted overall and renal survival already at 3 months after the start of therapy. Importantly, a parallel Italian study validated this new hematologic remission parameter. The outcome of prospective clinical trials might be adversely influenced by exclusion of the favorable clinical subgroup with an initial dFLC <50 mg/L. We propose the appreciation of dFLC in hematologic response assessment for all patients with a baseline dFLC >20 mg/L.
000128019 536__ $$0G:(DE-HGF)POF3-319H$$a319H - Addenda (POF3-319H)$$cPOF3-319H$$fPOF III$$x0
000128019 588__ $$aDataset connected to CrossRef, PubMed,
000128019 650_7 $$2NLM Chemicals$$aImmunoglobulin Light Chains
000128019 7001_ $$0P:(DE-He78)c741dc7f974390ad4310349f29aac40b$$aBochtler, Tilmann$$b1
000128019 7001_ $$aKimmich, Christoph$$b2
000128019 7001_ $$0P:(DE-He78)ecb33fb615e08035fdcefcaebfdff8f0$$aBecker, Natalia$$b3$$udkfz
000128019 7001_ $$aJauch, Anna$$b4
000128019 7001_ $$0P:(DE-He78)a1aa959d47e3e026abe157a8adf24b96$$aGoldschmidt, Hartmut$$b5
000128019 7001_ $$aHo, Anthony D$$b6
000128019 7001_ $$aHegenbart, Ute$$b7
000128019 7001_ $$aSchönland, Stefan O$$b8
000128019 773__ $$0PERI:(DE-600)1468538-3$$a10.1182/blood-2017-02-767475$$gVol. 130, no. 5, p. 632 - 642$$n5$$p632 - 642$$tBlood$$v130$$x1528-0020$$y2017
000128019 909CO $$ooai:inrepo02.dkfz.de:128019$$pVDB
000128019 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)c741dc7f974390ad4310349f29aac40b$$aDeutsches Krebsforschungszentrum$$b1$$kDKFZ
000128019 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)ecb33fb615e08035fdcefcaebfdff8f0$$aDeutsches Krebsforschungszentrum$$b3$$kDKFZ
000128019 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)a1aa959d47e3e026abe157a8adf24b96$$aDeutsches Krebsforschungszentrum$$b5$$kDKFZ
000128019 9131_ $$0G:(DE-HGF)POF3-319H$$1G:(DE-HGF)POF3-310$$2G:(DE-HGF)POF3-300$$3G:(DE-HGF)POF3$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lKrebsforschung$$vAddenda$$x0
000128019 9141_ $$y2017
000128019 915__ $$0StatID:(DE-HGF)0100$$2StatID$$aJCR$$bBLOOD : 2015
000128019 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS
000128019 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline
000128019 915__ $$0StatID:(DE-HGF)0310$$2StatID$$aDBCoverage$$bNCBI Molecular Biology Database
000128019 915__ $$0StatID:(DE-HGF)0600$$2StatID$$aDBCoverage$$bEbsco Academic Search
000128019 915__ $$0StatID:(DE-HGF)0030$$2StatID$$aPeer Review$$bASC
000128019 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bThomson Reuters Master Journal List
000128019 915__ $$0StatID:(DE-HGF)0110$$2StatID$$aWoS$$bScience Citation Index
000128019 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection
000128019 915__ $$0StatID:(DE-HGF)0111$$2StatID$$aWoS$$bScience Citation Index Expanded
000128019 915__ $$0StatID:(DE-HGF)1110$$2StatID$$aDBCoverage$$bCurrent Contents - Clinical Medicine
000128019 915__ $$0StatID:(DE-HGF)1030$$2StatID$$aDBCoverage$$bCurrent Contents - Life Sciences
000128019 915__ $$0StatID:(DE-HGF)1050$$2StatID$$aDBCoverage$$bBIOSIS Previews
000128019 915__ $$0StatID:(DE-HGF)9910$$2StatID$$aIF >= 10$$bBLOOD : 2015
000128019 9201_ $$0I:(DE-He78)G330-20160331$$kG330$$lKKE Molekulare Hämatologie/Onkologie$$x0
000128019 9201_ $$0I:(DE-He78)V964-20160331$$kV964$$lNCT-KliHD$$x1
000128019 9201_ $$0I:(DE-He78)C060-20160331$$kC060$$lBiostatistik$$x2
000128019 980__ $$ajournal
000128019 980__ $$aVDB
000128019 980__ $$aI:(DE-He78)G330-20160331
000128019 980__ $$aI:(DE-He78)V964-20160331
000128019 980__ $$aI:(DE-He78)C060-20160331
000128019 980__ $$aUNRESTRICTED