Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia

  1. Zielen, Stefan
  2. Duecker, Ruth Pia
  3. Woelke, Sandra
  4. Donath, Helena
  5. Bakhtiar, Sharhzad
  6. Buecker, Aileen
  7. Kreyenberg, Hermann
  8. Huenecke, Sabine
  9. Bader, Peter
  10. Mahlaoui, Nizar
  11. Ehl, Stephan
  12. El-Helou, Sabine M.
  13. Pietrucha, Barbara
  14. Plebani, Alessandro
  15. van der Flier, Michiel
  16. van Aerde, Koen
  17. Kilic, Sara S.
  18. Reda, Shereen M.
  19. Kostyuchenko, Larysa
  20. McDermott, Elizabeth
  21. Galal, Nermeen
  22. Pignata, Claudio
  23. Pérez, Juan Luis Santos
  24. Laws, Hans-Juergen
  25. Niehues, Tim
  26. Kutukculer, Necil
  27. Seidel, Markus G.
  28. Marques, Laura
  29. Ciznar, Peter
  30. Edgar, John David M.
  31. Soler-Palacín, Pere
  32. von Bernuth, Horst
  33. Krueger, Renate
  34. Meyts, Isabelle
  35. Baumann, Ulrich
  36. Kanariou, Maria
  37. Grimbacher, Bodo
  38. Hauck, Fabian
  39. Graf, Dagmar
  40. Granado, Luis Ignacio Gonzalez
  41. Prader, Seraina
  42. Reisli, Ismail
  43. Slatter, Mary
  44. Rodríguez-Gallego, Carlos
  45. Arkwright, Peter D.
  46. Bethune, Claire
  47. Deripapa, Elena
  48. Sharapova, Svetlana O.
  49. Lehmberg, Kai
  50. Davies, E. Graham
  51. Schuetz, Catharina
  52. Kindle, Gerhard
  53. Schubert, Ralf

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1000 Titel
  • Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia
1000 Autor/in
  1. Zielen, Stefan |
  2. Duecker, Ruth Pia |
  3. Woelke, Sandra |
  4. Donath, Helena |
  5. Bakhtiar, Sharhzad |
  6. Buecker, Aileen |
  7. Kreyenberg, Hermann |
  8. Huenecke, Sabine |
  9. Bader, Peter |
  10. Mahlaoui, Nizar |
  11. Ehl, Stephan |
  12. El-Helou, Sabine M. |
  13. Pietrucha, Barbara |
  14. Plebani, Alessandro |
  15. van der Flier, Michiel |
  16. van Aerde, Koen |
  17. Kilic, Sara S. |
  18. Reda, Shereen M. |
  19. Kostyuchenko, Larysa |
  20. McDermott, Elizabeth |
  21. Galal, Nermeen |
  22. Pignata, Claudio |
  23. Pérez, Juan Luis Santos |
  24. Laws, Hans-Juergen |
  25. Niehues, Tim |
  26. Kutukculer, Necil |
  27. Seidel, Markus G. |
  28. Marques, Laura |
  29. Ciznar, Peter |
  30. Edgar, John David M. |
  31. Soler-Palacín, Pere |
  32. von Bernuth, Horst |
  33. Krueger, Renate |
  34. Meyts, Isabelle |
  35. Baumann, Ulrich |
  36. Kanariou, Maria |
  37. Grimbacher, Bodo |
  38. Hauck, Fabian |
  39. Graf, Dagmar |
  40. Granado, Luis Ignacio Gonzalez |
  41. Prader, Seraina |
  42. Reisli, Ismail |
  43. Slatter, Mary |
  44. Rodríguez-Gallego, Carlos |
  45. Arkwright, Peter D. |
  46. Bethune, Claire |
  47. Deripapa, Elena |
  48. Sharapova, Svetlana O. |
  49. Lehmberg, Kai |
  50. Davies, E. Graham |
  51. Schuetz, Catharina |
  52. Kindle, Gerhard |
  53. Schubert, Ralf |
1000 Erscheinungsjahr 2021
1000 Publikationstyp
  1. Artikel |
1000 Online veröffentlicht
  • 2021-09-03
1000 Erschienen in
1000 Quellenangabe
  • 41(8):1878-1892
1000 Copyrightjahr
  • 2021
1000 Lizenz
1000 Verlagsversion
  • https://doi.org/10.1007/s10875-021-01090-8 |
  • https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8604875/ |
1000 Publikationsstatus
1000 Sprache der Publikation
1000 Abstract/Summary
  • Patients with ataxia-telangiectasia (A-T) suffer from progressive cerebellar ataxia, immunodeficiency, respiratory failure, and cancer susceptibility. From a clinical point of view, A-T patients with IgA deficiency show more symptoms and may have a poorer prognosis. In this study, we analyzed mortality and immunity data of 659 A-T patients with regard to IgA deficiency collected from the European Society for Immunodeficiencies (ESID) registry and from 66 patients with classical A-T who attended at the Frankfurt Goethe-University between 2012 and 2018. We studied peripheral B- and T-cell subsets and T-cell repertoire of the Frankfurt cohort and survival rates of all A-T patients in the ESID registry. Patients with A-T have significant alterations in their lymphocyte phenotypes. All subsets (CD3, CD4, CD8, CD19, CD4/CD45RA, and CD8/CD45RA) were significantly diminished compared to standard values. Patients with IgA deficiency (n = 35) had significantly lower lymphocyte counts compared to A-T patients without IgA deficiency (n = 31) due to a further decrease of naïve CD4 T-cells, central memory CD4 cells, and regulatory T-cells. Although both patient groups showed affected TCR-ß repertoires compared to controls, no differences could be detected between patients with and without IgA deficiency. Overall survival of patients with IgA deficiency was significantly diminished. For the first time, our data show that patients with IgA deficiency have significantly lower lymphocyte counts and subsets, which are accompanied with reduced survival, compared to A-T patients without IgA deficiency. IgA, a simple surrogate marker, is indicating the poorest prognosis for classical A-T patients. Both non-interventional clinical trials were registered at clinicaltrials.gov 2012 (Susceptibility to infections in ataxia-telangiectasia; NCT02345135) and 2017 (Susceptibility to Infections, tumor risk and liver disease in patients with ataxia-telangiectasia; NCT03357978).
1000 Sacherschließung
lokal Ataxia Telangiectasia/mortality [MeSH]
lokal Immunoglobulin A/blood [MeSH]
lokal T-Lymphocyte Subsets/immunology [MeSH]
lokal IgA deficiency
lokal Lymphopenia
lokal Original Article
lokal Infant [MeSH]
lokal Mortality
lokal Male [MeSH]
lokal Ataxia Telangiectasia/immunology [MeSH]
lokal Child [MeSH]
lokal Ataxia-telangiectasia
lokal Adolescent [MeSH]
lokal Female [MeSH]
lokal IgG Deficiency/immunology [MeSH]
lokal Immunodeficiency
lokal Immunoglobulin M/blood [MeSH]
lokal Immunoglobulins
lokal Adult [MeSH]
lokal B-Lymphocytes/immunology [MeSH]
lokal Lymphocyte Count [MeSH]
lokal Humans [MeSH]
lokal Middle Aged [MeSH]
lokal IgA Deficiency/immunology [MeSH]
lokal Young Adult [MeSH]
lokal IgA Deficiency/mortality [MeSH]
lokal Immunoglobulin G/blood [MeSH]
lokal IgG Deficiency/mortality [MeSH]
lokal Child, Preschool [MeSH]
1000 Liste der Beteiligten
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