Allogeneic hematopoietic stem cell transplantation for adult HLH: a retrospective study by the chronic malignancies and inborn errors working parties of EBMT.
(2022) Bone Marrow Transplantation — Vol. 57, n° 5, p. 817-823 (2022)
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- Authors
Machowicz, Rafal 
Suarez, Felipe Wiktor-Jedrzejczak, Wieslaw
- 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.
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Citations
Machowicz, R., Suarez, F., Wiktor-Jedrzejczak, W., Eikema, D.-J., de Wreede, L. C., 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., Díez-Martín, J. L., Bonifazi, F., et al. (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 (Original work published 2022)