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  • Machowicz, RafalMed Univ Warsaw, Dept Hematol Transplantat & Internal Med, Warsaw, Poland (author)

Allogeneic hematopoietic stem cell transplantation for adult HLH : a retrospective study by the chronic malignancies and inborn errors working parties of EBMT

  • Article/chapterEnglish2022

Publisher, publication year, extent ...

  • 2022-03-24
  • Springer Nature,2022
  • printrdacarrier

Numbers

  • LIBRIS-ID:oai:DiVA.org:uu-485994
  • https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-485994URI
  • https://doi.org/10.1038/s41409-022-01634-5DOI
  • http://kipublications.ki.se/Default.aspx?queryparsed=id:149140030URI

Supplementary language notes

  • Language:English
  • Summary in:English

Part of subdatabase

Classification

  • Subject category:ref swepub-contenttype
  • Subject category:art swepub-publicationtype

Notes

  • Hemophagocytic lymphohistiocytosis (HLH; hemophagocytic syndrome) is a rare syndrome of potentially fatal, uncontrolled hyperinflammation. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is indicated in primary, recurrent or progressive HLH, but information about its outcomes in the adult population is limited. We obtained data about 87 adult (>= 18 years of age) patients retrospectively reported to the EBMT. The median survival time was 13.9 months. The three and five-year overall survival (OS) was 44% (95% CI 33-54%). Among 39 patients with a follow-up longer than 15 months, only three died. Relapse rate was 21% (95% CI 13-30%), while NRM reached 36% (95% CI 25-46%). Younger patients (<30 years of age) had better prognosis, with an OS of 59% (95% CI 45-73%) at three and five years vs 23% (95% CI 8-37%) for older ones. No difference in survival between reduced and myeloablative conditioning was found. To our knowledge, this is the largest report of adult HLH patients who underwent allo-HSCT. Patients who survive the first period after this procedure can expect a long disease-free survival. Both reduced intensity and myeloablative conditioning have therapeutic potential in adult HLH.

Subject headings and genre

Added entries (persons, corporate bodies, meetings, titles ...)

  • Suarez, FelipeUniv Paris, Hop Necker Enfants Malad, AP HP,Ctr Univ Paris, Serv Hematol Adultes,Inst Imagine INSERM UMR 1163, Paris, France.;Univ Paris, Paris, France (author)
  • Wiktor-Jedrzejczak, WieslawMed Univ Warsaw, Dept Hematol Transplantat & Internal Med, Warsaw, Poland (author)
  • Eikema, Diderik-JanEBMT Stat Unit, Leiden, Netherlands (author)
  • de Wreede, Liesbeth C.LUMC, Dept Biomed Data Sci, Leiden, Netherlands (author)
  • Blok, Henric-JanEBMT Data Off Leiden, Leiden, Netherlands (author)
  • Isaksson, CeciliaUmeå Univ Hosp, Umeå, Sweden (author)
  • Einsele, HermannUniv Klinikum Wurzburg, Wurzburg, Germany (author)
  • Poire, XavierClin Univ St Luc, Brussels, Belgium (author)
  • van Dorp, SuzanneRadboud Univ Nijmegen, Med Ctr, Nijmegen, Netherlands (author)
  • Nikolousis, EmmanouilBirmingham Heartlands Hosp, Birmingham, W Midlands, England (author)
  • Johansson, Jan-ErikSahlgrens Univ Hosp, Gothenburg, Sweden (author)
  • Kobbe, GuidoHeinrich Heine Univ Dusseldorf, Univ Hosp Dusseldorf, Med Fac, Dusseldorf, Germany (author)
  • Zecca, MarcoFdn IRCCS Policlin San Matteo, Pediat Hematol Oncol, Pavia, Italy (author)
  • Arnold, RenateCharite Univ Med Berlin, Berlin, Germany (author)
  • Gerbitz, ArminCharite Univ Med Berlin, Berlin, Germany (author)
  • Finke, JuergenUniv Freiburg, Freiburg, Germany (author)
  • Diez-Martin, Jose LuisUniv Complutense, Hosp Gen Univ Gregorio Maranon, Inst Invest Sanitarias Gregorio Maranon, Hematol Dept, Madrid, Spain (author)
  • Bonifazi, FrancescaIRCCS Azienda Osped Univ Bologna, Bologna, Italy (author)
  • McQuaker, GrantQueen Elizabeth Univ Hosp, Glasgow, Lanark, Scotland (author)
  • Lenhoff, StigSkanes Univ Hosp, Lund, Sweden (author)
  • Rohrlich, Pierre-SimonUniv Cote dAzur, Dept Hematol, CHU Nice, Nice, France (author)
  • Theobald, MatthiasUniv Canc Ctr, Univ Med Ctr, Dept Hematol Med Oncol & Pneumol, Mainz, Germany (author)
  • Ljungman, PerKarolinska Institutet (author)
  • Collin, MatthewFreeman Rd Hosp, Newcastle Upon Tyne, Tyne & Wear, England (author)
  • Albert, Michael H.LMU, Dr von Hauner Childrens Hosp, Dept Pediat, Univ Hosp, Munich, Germany (author)
  • Ehninger, GerhardUniv Klinikum Dresden, Dresden, Germany (author)
  • Carlson, KristinaUppsala universitet,Hematologi,Univ Hosp, Uppsala, Sweden(Swepub:uu)krica965 (author)
  • Halaburda, KazimierzInst Hematol & Transfus Med, Warsaw, Poland (author)
  • Lehmberg, KaiUniv Hosp Eppendorf, Hamburg, Germany (author)
  • Schoenland, StefanHeidelberg Univ, Heidelberg, Germany (author)
  • Yakoub-Agha, IbrahimUniv Lille, Infinite, INSERM U1286, CHU Lille, F-59000 Lille, France (author)
  • Gennery, Andrew R.Newcastle Univ, Translat & Clin Res Inst, Newcastle Upon Tyne, Tyne & Wear, England.;Great North Childrens Hosp Newcastle Upon Tyne, Dept Paediat Immunol HSCT, Newcastle Upon Tyne, Tyne & Wear, England (author)
  • Lankester, Arjan C.Leiden Univ, Willem Alexander Childrens Hosp, Paediat Stem Cell Transplantat Program, Med Ctr, Leiden, Netherlands (author)
  • Kroeger, NicolausUniv Hosp Eppendorf, Hamburg, Germany (author)
  • Med Univ Warsaw, Dept Hematol Transplantat & Internal Med, Warsaw, PolandUniv Paris, Hop Necker Enfants Malad, AP HP,Ctr Univ Paris, Serv Hematol Adultes,Inst Imagine INSERM UMR 1163, Paris, France.;Univ Paris, Paris, France (creator_code:org_t)

Related titles

  • In:Bone Marrow Transplantation: Springer Nature57:5, s. 817-8230268-33691476-5365

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