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

Autor: Rafal Machowicz, Felipe Suarez, Wieslaw Wiktor-Jedrzejczak, Diderik-Jan Eikema, Liesbeth C. de Wreede, Henric-Jan Blok, Cecilia Isaksson, Hermann Einsele, Xavier Poiré, Suzanne van Dorp, Emmanouil Nikolousis, Jan-Erik Johansson, Guido Kobbe, Marco Zecca, Renate Arnold, Armin Gerbitz, Jürgen Finke, Jose Luis Díez-Martín, Francesca Bonifazi, Grant McQuaker, Stig Lenhoff, Pierre-Simon Rohrlich, Matthias Theobald, Per Ljungman, Matthew Collin, Michael H. Albert, Gerhard Ehninger, Kristina Carlson, Kazimierz Halaburda, Kai Lehmberg, Stefan Schönland, Ibrahim Yakoub-Agha, Andrew R. Gennery, Arjan C. Lankester, Nicolaus Kröger
Přispěvatelé: UCL - SSS/IREC/SLUC - Pôle St.-Luc, UCL - (SLuc) Centre du cancer, UCL - (SLuc) Service d'hématologie
Rok vydání: 2022
Předmět:
Zdroj: Bone Marrow Transplantation, 57, 817-823
Bone Marrow Transplantation, 57, 5, pp. 817-823
Bone marrow transplantation, Vol. 57, no.5, p. 817-823 (2022)
Bone Marrow Transplantation, 57(5), 817-823. SPRINGERNATURE
ISSN: 1476-5365
0268-3369
DOI: 10.1038/s41409-022-01634-5
Popis: Contains fulltext : 251601.pdf (Publisher’s version ) (Closed access) 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 (
Databáze: OpenAIRE
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