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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

Lookup NU author(s): Professor Matthew CollinORCiD, Professor Andrew GenneryORCiD

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Abstract

© 2022, The Author(s), under exclusive licence to Springer Nature Limited. 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.

Publication metadata

Author(s): Machowicz R, Suarez F, Wiktor-Jedrzejczak W, Eikema D-J, de Wreede LC, Blok H-J, Isaksson C, Einsele H, Poire X, van Dorp S, Nikolousis E, Johansson J-E, Kobbe G, Zecca M, Arnold R, Gerbitz A, Finke J, Diez-Martin JL, Bonifazi F, McQuaker G, Lenhoff S, Rohrlich P-S, Theobald M, Ljungman P, Collin M, Albert MH, Ehninger G, Carlson K, Halaburda K, Lehmberg K, Schonland S, Yakoub-Agha I, Gennery AR, Lankester AC, Kroger N

Publication type: Article

Publication status: Published

Journal: Bone Marrow Transplantation

Year: 2022

Volume: 57

Pages: 817-823

Online publication date: 24/03/2022

Acceptance date: 02/03/2022

ISSN (print): 0268-3369

ISSN (electronic): 1476-5365

Publisher: Nature Publishing Group

URL: https://doi.org/10.1038/s41409-022-01634-5

DOI: 10.1038/s41409-022-01634-5

PubMed id: 35332305

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