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@ARTICLE{Dittrich:128019,
      author       = {T. Dittrich and T. Bochtler$^*$ and C. Kimmich and N.
                      Becker$^*$ and A. Jauch and H. Goldschmidt$^*$ and A. D. Ho
                      and U. Hegenbart and S. O. Schönland},
      title        = {{AL} amyloidosis patients with low amyloidogenic free light
                      chain levels at first diagnosis have an excellent
                      prognosis.},
      journal      = {Blood},
      volume       = {130},
      number       = {5},
      issn         = {1528-0020},
      address      = {Stanford, Calif.},
      publisher    = {HighWire Press},
      reportid     = {DKFZ-2017-04041},
      pages        = {632 - 642},
      year         = {2017},
      abstract     = {The 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.},
      keywords     = {Immunoglobulin Light Chains (NLM Chemicals)},
      cin          = {G330 / V964 / C060},
      ddc          = {610},
      cid          = {I:(DE-He78)G330-20160331 / I:(DE-He78)V964-20160331 /
                      I:(DE-He78)C060-20160331},
      pnm          = {319H - Addenda (POF3-319H)},
      pid          = {G:(DE-HGF)POF3-319H},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:28550043},
      doi          = {10.1182/blood-2017-02-767475},
      url          = {https://inrepo02.dkfz.de/record/128019},
}