1. Allogeneic hematopoietic stem cell transplantation for adult HLH: a retrospective study by the chronic malignancies and inborn errors working parties of EBMT
- Author
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Machowicz, R., Suarez, F., Wiktor-Jedrzejczak, W., Eikema, D.J., Wreede, L.C. de, Blok, H.J., Isaksson, C., Einsele, H., Poiré, X., Dorp, S. van, Nikolousis, E., Johansson, J.E., Kobbe, G., Zecca, M., Arnold, R., Gerbitz, A., Finke, J., Díez-Martín, J.L., Bonifazi, F., McQuaker, G., Lenhoff, S., Rohrlich, P.S., Theobald, M., Ljungman, P., Collin, M., Albert, M.H., Ehninger, G., Carlson, K., Halaburda, K., Lehmberg, K., Schönland, S., Yakoub-Agha, I., Gennery, A.R., Lankester, A.C., Kröger, N., Machowicz, R., Suarez, F., Wiktor-Jedrzejczak, W., Eikema, D.J., Wreede, L.C. de, Blok, H.J., Isaksson, C., Einsele, H., Poiré, X., Dorp, S. van, Nikolousis, E., Johansson, J.E., Kobbe, G., Zecca, M., Arnold, R., Gerbitz, A., Finke, J., Díez-Martín, J.L., Bonifazi, F., McQuaker, G., Lenhoff, S., Rohrlich, P.S., Theobald, M., Ljungman, P., Collin, M., Albert, M.H., Ehninger, G., Carlson, K., Halaburda, K., Lehmberg, K., Schönland, S., Yakoub-Agha, I., Gennery, A.R., Lankester, A.C., and Kröger, N.
- Abstract
Item does not contain fulltext, 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.
- Published
- 2022