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Allogeneic hematopoietic stem cell transplantation for adult HLH: a retrospective study by the chronic malignancies and inborn errors working parties of EBMT
Med Univ Warsaw, Dept Hematol Transplantat & Internal Med, Warsaw, Poland.ORCID iD: 0000-0002-2077-6610
Univ Paris, Hop Necker Enfants Malad, AP HP,Ctr Univ Paris, Serv Hematol Adultes,Inst Imagine INSERM UMR 1163, Paris, France.;Univ Paris, Paris, France.ORCID iD: 0000-0002-3537-9052
Med Univ Warsaw, Dept Hematol Transplantat & Internal Med, Warsaw, Poland.
EBMT Stat Unit, Leiden, Netherlands.
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2022 (English)In: Bone Marrow Transplantation, ISSN 0268-3369, E-ISSN 1476-5365, Vol. 57, no 5, p. 817-823Article in journal (Refereed) Published
Abstract [en]

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.

Place, publisher, year, edition, pages
Springer Nature, 2022. Vol. 57, no 5, p. 817-823
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Hematology
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URN: urn:nbn:se:uu:diva-485994DOI: 10.1038/s41409-022-01634-5ISI: 000772960600002PubMedID: 35332305OAI: oai:DiVA.org:uu-485994DiVA, id: diva2:1702847
Available from: 2022-10-11 Created: 2022-10-11 Last updated: 2022-10-11Bibliographically approved

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Publisher's full textPubMedhttps://www.nature.com/articles/s41409-022-01634-5#article-info

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Carlson, Kristina

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