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Allogeneic hematopo...
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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 ...
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2022-03-24
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Springer Nature,2022
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printrdacarrier
Numbers
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LIBRIS-ID:oai:DiVA.org:uu-485994
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https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-485994URI
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https://doi.org/10.1038/s41409-022-01634-5DOI
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http://kipublications.ki.se/Default.aspx?queryparsed=id:149140030URI
Supplementary language notes
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Language:English
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Summary in:English
Part of subdatabase
Classification
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Subject category:ref swepub-contenttype
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Subject category:art swepub-publicationtype
Notes
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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 ...)
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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)
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Wiktor-Jedrzejczak, WieslawMed Univ Warsaw, Dept Hematol Transplantat & Internal Med, Warsaw, Poland
(author)
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Eikema, Diderik-JanEBMT Stat Unit, Leiden, Netherlands
(author)
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de Wreede, Liesbeth C.LUMC, Dept Biomed Data Sci, Leiden, Netherlands
(author)
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Blok, Henric-JanEBMT Data Off Leiden, Leiden, Netherlands
(author)
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Isaksson, CeciliaUmeå Univ Hosp, Umeå, Sweden
(author)
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Einsele, HermannUniv Klinikum Wurzburg, Wurzburg, Germany
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Poire, XavierClin Univ St Luc, Brussels, Belgium
(author)
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van Dorp, SuzanneRadboud Univ Nijmegen, Med Ctr, Nijmegen, Netherlands
(author)
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Nikolousis, EmmanouilBirmingham Heartlands Hosp, Birmingham, W Midlands, England
(author)
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Johansson, Jan-ErikSahlgrens Univ Hosp, Gothenburg, Sweden
(author)
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Kobbe, GuidoHeinrich Heine Univ Dusseldorf, Univ Hosp Dusseldorf, Med Fac, Dusseldorf, Germany
(author)
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Zecca, MarcoFdn IRCCS Policlin San Matteo, Pediat Hematol Oncol, Pavia, Italy
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Arnold, RenateCharite Univ Med Berlin, Berlin, Germany
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Gerbitz, ArminCharite Univ Med Berlin, Berlin, Germany
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Finke, JuergenUniv Freiburg, Freiburg, Germany
(author)
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Diez-Martin, Jose LuisUniv Complutense, Hosp Gen Univ Gregorio Maranon, Inst Invest Sanitarias Gregorio Maranon, Hematol Dept, Madrid, Spain
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Bonifazi, FrancescaIRCCS Azienda Osped Univ Bologna, Bologna, Italy
(author)
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McQuaker, GrantQueen Elizabeth Univ Hosp, Glasgow, Lanark, Scotland
(author)
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Lenhoff, StigSkanes Univ Hosp, Lund, Sweden
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Rohrlich, Pierre-SimonUniv Cote dAzur, Dept Hematol, CHU Nice, Nice, France
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Theobald, MatthiasUniv Canc Ctr, Univ Med Ctr, Dept Hematol Med Oncol & Pneumol, Mainz, Germany
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Ljungman, PerKarolinska Institutet
(author)
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Collin, MatthewFreeman Rd Hosp, Newcastle Upon Tyne, Tyne & Wear, England
(author)
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Albert, Michael H.LMU, Dr von Hauner Childrens Hosp, Dept Pediat, Univ Hosp, Munich, Germany
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Ehninger, GerhardUniv Klinikum Dresden, Dresden, Germany
(author)
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Carlson, KristinaUppsala universitet,Hematologi,Univ Hosp, Uppsala, Sweden(Swepub:uu)krica965
(author)
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Halaburda, KazimierzInst Hematol & Transfus Med, Warsaw, Poland
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Lehmberg, KaiUniv Hosp Eppendorf, Hamburg, Germany
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Schoenland, StefanHeidelberg Univ, Heidelberg, Germany
(author)
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Yakoub-Agha, IbrahimUniv Lille, Infinite, INSERM U1286, CHU Lille, F-59000 Lille, France
(author)
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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)
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Lankester, Arjan C.Leiden Univ, Willem Alexander Childrens Hosp, Paediat Stem Cell Transplantat Program, Med Ctr, Leiden, Netherlands
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Kroeger, NicolausUniv Hosp Eppendorf, Hamburg, Germany
(author)
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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
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In:Bone Marrow Transplantation: Springer Nature57:5, s. 817-8230268-33691476-5365
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Machowicz, Rafal
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Suarez, Felipe
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Wiktor-Jedrzejcz ...
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Eikema, Diderik- ...
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de Wreede, Liesb ...
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Blok, Henric-Jan
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Isaksson, Cecili ...
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Einsele, Hermann
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Poire, Xavier
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van Dorp, Suzann ...
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Nikolousis, Emma ...
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Johansson, Jan-E ...
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Kobbe, Guido
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Zecca, Marco
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Arnold, Renate
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Gerbitz, Armin
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Finke, Juergen
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Diez-Martin, Jos ...
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Bonifazi, France ...
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McQuaker, Grant
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Lenhoff, Stig
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Rohrlich, Pierre ...
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Theobald, Matthi ...
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Ljungman, Per
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Collin, Matthew
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Albert, Michael ...
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Ehninger, Gerhar ...
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Carlson, Kristin ...
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Halaburda, Kazim ...
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Lehmberg, Kai
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Schoenland, Stef ...
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Yakoub-Agha, Ibr ...
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Gennery, Andrew ...
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Lankester, Arjan ...
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Kroeger, Nicolau ...
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