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

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.

• Authors: 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
• Journal: Bone marrow transplantation (Bone Marrow Transplant) Vol. 57 Issue 5 Pg. 817-823 (05 2022) ISSN: 1476-5365 [Electronic] England
• PMID: 35332305 (Publication Type: Journal Article)
• Copyright: © 2022. The Author(s), under exclusive licence to Springer Nature Limited.

Topics

• Adult
• Child, Preschool
• Graft vs Host Disease
• Hematopoietic Stem Cell Transplantation (methods)
• Humans
• Lymphohistiocytosis, Hemophagocytic (pathology, therapy)
• Neoplasms
• Retrospective Studies
• Transplantation Conditioning (methods)