Title :

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

Author(s) :

Machowicz, R. [Auteur]
Suarez, Felipe [Auteur]
Imagine - Institut des maladies génétiques (IHU) [Imagine - U1163]
Mécanismes cellulaires et moléculaires des désordres hématologiques et implications thérapeutiques = Molecular mechanisms of hematological disorders and therapeutic implications [ERL 8254]
Wiktor-Jedrzejczak, W. [Auteur]
Eikema, D. J. [Auteur]
De Wreede, L. C. [Auteur]
Blok, H. J. [Auteur]
Isaksson, C. [Auteur]
Einsele, H. [Auteur]
Poiré, X. [Auteur]
Van Dorp, S. [Auteur]
Nikolousis, E. [Auteur]
Johansson, J. E. [Auteur]
Kobbe, G. [Auteur]
Zecca, M. [Auteur]
Arnold, R. [Auteur]
Gerbitz, A. [Auteur]
Finke, J. [Auteur]
Díez-Martín, J. L. [Auteur]
Bonifazi, F. [Auteur]
Mcquaker, G. [Auteur]
Lenhoff, S. [Auteur]
Rohrlich, Pierre-Simon [Auteur]
Centre Hospitalier Universitaire de Nice [CHU Nice]
Theobald, M. [Auteur]
Ljungman, P. [Auteur]
Collin, M. [Auteur]
Albert, M. H. [Auteur]
Ehninger, G. [Auteur]
Carlson, K. [Auteur]
Halaburda, K. [Auteur]
Lehmberg, K. [Auteur]
Schönland, S. [Auteur]
Yakoub-Agha, Ibrahim [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Gennery, A. R. [Auteur]
Lankester, A. C. [Auteur]
Kröger, N. [Auteur]

Journal title :

Bone Marrow Transplantation

Abbreviated title :

Bone Marrow Transplant

Volume number :

57

Pages :

817–823

Publication date :

2022-03-27

ISSN :

1476-5365

HAL domain(s) :

Sciences du Vivant [q-bio]

English 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 ...
Show more >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.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2024-01-12T01:59:28Z
2024-03-14T10:18:18Z