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@ARTICLE{Ruzicka:301720,
      author       = {M. Ruzicka and T. Wimmer and H.-J. Stemmler and S.-S.
                      Stecher and H. Schulze-Koops and F. Hauck and M.
                      Subklewe$^*$ and M. von Bergwelt-Baildon$^*$ and K.
                      Spiekermann$^*$},
      title        = {{C}linical features, course, and risk factors of
                      infection-associated secondary hemophagocytic
                      lymphohistiocytosis.},
      journal      = {Infection},
      volume       = {nn},
      issn         = {0300-8126},
      address      = {München},
      publisher    = {Urban $\&$ Vogel},
      reportid     = {DKFZ-2025-01112},
      pages        = {nn},
      year         = {2025},
      note         = {epub},
      abstract     = {Hemophagocytic lymphohistiocytosis (HLH) is an orphan
                      disease characterized by excessive inflammation and poor
                      outcome. We sought to further characterize clinical
                      features, courses, and risk factors of secondary HLH (sHLH)
                      triggered by infection (iHLH). 28 $(43.1\%)$ of 65 adult
                      sHLH cases treated at our hospital from 2012-2024 were
                      infection-associated. iHLH patients were mostly male
                      $(71.4\%).$ Infectious agents most frequently detected were
                      EBV $(57.1\%)$ and leishmania $(14.3\%).$ The median time to
                      diagnosis was 13 [6.0;24.8] days. iHLH patients had a
                      mortality rate of $39.3\%$ (median follow-up time: 735
                      [336;1140] days), worse survival than patients with
                      autoimmune-triggered (hazard ratio: 3.33 (1.01-11.10), p =
                      0.049), and better survival than patients with
                      paraneoplastic HLH (hazard ratio: 0.19 (0.10-0.84), p =
                      0.002). Elevated levels of soluble interleukin-2 receptor
                      (sIL2R; > 6,000 I/U), low thrombocyte counts (< 40 G/l), and
                      a history of malignant disease were associated with adverse
                      outcomes. Protracted time to diagnosis was associated with
                      severe disease courses and with leishmaniosis. Further,
                      sIL2R levels correlated positively with prolonged aPTT and
                      thrombocytopenia, and hypertriglyceridemia with elevated
                      INRs. Patients with an elevated sIL2R:ferritin ratio were
                      more likely to have a history of malignant comorbidities.
                      Taken together, sIL2R, thrombocytopenia, and a history of
                      malignant disease are important prognostic factors of iHLH.
                      Patients with high sIL2R levels or hypertriglyceridemia may
                      be at higher risk of bleeding, and patients with elevated
                      sIL2R:ferritin ratios should be assessed for possible
                      malignant comorbidities. Lastly, increased awareness of the
                      disease and newly emerging pathogens (i.e. leishmania) may
                      shorten the time to diagnosis, and thus reduce severe
                      courses of iHLH.},
      keywords     = {Hemophagocytic lymphohistiocytosis (Other) /
                      Infection-associated HLH (Other) / Prognostic factors of HLH
                      (Other) / Risk factors of HLH (Other) / Secondary HLH
                      (Other)},
      cin          = {MU01},
      ddc          = {610},
      cid          = {I:(DE-He78)MU01-20160331},
      pnm          = {899 - ohne Topic (POF4-899)},
      pid          = {G:(DE-HGF)POF4-899},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:40425997},
      doi          = {10.1007/s15010-025-02559-z},
      url          = {https://inrepo02.dkfz.de/record/301720},
}