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

Machowicz R, Suarez F, Wiktor-Jedrzejczak W, Eikema DJ, De Wreede LC, Blok HJ, Isaksson C, Einsele H, Poire X, Van Dorp S, Nikolousis E, Johansson JE, Kobbe G, Zecca M, Arnold R, Gerbitz A, Finke J, Diez-Martin JL, Bonifazi F, Mcquaker G, Lenhoff S, Rohrlich PS, Theobald M, Ljungman P, Collin M, Albert MH, Ehninger G, Carlson K, Halaburda K, Lehmberg K, Schoenland S, Yakoub-Agha I, Gennery AR, Lankester AC, Kroeger N (2022)

Publication Type: Journal article

Publication year: 2022

Journal

Book Volume: 57

Pages Range: 817-823

Journal Issue: 5

DOI: 10.1038/s41409-022-01634-5

Abstract

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.

Involved external institutions

Wrocław Medical University / Uniwersytet Medyczny we Wrocławiu Poland (PL) Université de Paris France (FR) European Society for Blood and Marrow Transplantation EBMT Netherlands (NL) Leiden University Medical Center Netherlands (NL) Heartlands Hospital United Kingdom (GB) Policlinico San Matteo Pavia Fondazione IRCCS Italy (IT) Charité - Universitätsmedizin Berlin Germany (DE) Universidad Complutense de Madrid (UCM) Spain (ES) Skåne University Hospital / Skånes universitetssjukhus Sweden (SE) University of Côte d'Azur / Université Côte d'Azur France (FR) Universitätsmedizin der Johannes Gutenberg-Universität Mainz Germany (DE) Ludwig-Maximilians-Universität (LMU) Germany (DE) Universitätsklinikum Carl Gustav Carus Dresden Germany (DE) Universitätsklinikum Heidelberg Germany (DE) Universitätsklinikum Hamburg-Eppendorf (UKE) Germany (DE) Ruprecht-Karls-Universität Heidelberg Germany (DE) LILLE 1 University - Science and Technology France (FR) Leiden University Netherlands (NL) University Hospital of Umeå / Norrlands universitetssjukhus (NUS) Sweden (SE) Sahlgrenska University Hospital / Sahlgrenska Universitetssjukhuset Sweden (SE) Heinrich-Heine-Universität Düsseldorf Germany (DE) Albert-Ludwigs-Universität Freiburg Germany (DE) Ospedaliero Universitaria di Bologna Policlinico S.Orsola-Malpighi Italy (IT) Queen Elizabeth University Hospital United Kingdom (GB) Karolinska University Hospital / Karolinska Universitetssjukhuset Sweden (SE) Newcastle upon Tyne Hospitals NHS Foundation Trust United Kingdom (GB) Newcastle University United Kingdom (GB) Universitätsklinikum Würzburg Germany (DE) Cliniques universitaires Saint-Luc (CHU St-Luc) Belgium (BE) Radboud University Nijmegen Netherlands (NL) Institute of Hematology and Blood Transfusion / Instytut Hematologii i Transfuzjologi (IHiT) Poland (PL)

How to cite

APA:

Machowicz, R., Suarez, F., Wiktor-Jedrzejczak, W., Eikema, D.-J., De Wreede, L.C., Blok, H.-J.,... Kroeger, N. (2022). Allogeneic hematopoietic stem cell transplantation for adult HLH: a retrospective study by the chronic malignancies and inborn errors working parties of EBMT. Bone Marrow Transplantation, 57(5), 817-823. https://doi.org/10.1038/s41409-022-01634-5

MLA:

Machowicz, Rafal, et al. "Allogeneic hematopoietic stem cell transplantation for adult HLH: a retrospective study by the chronic malignancies and inborn errors working parties of EBMT." Bone Marrow Transplantation 57.5 (2022): 817-823.

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