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  • Felber, M. (author)

Targeted busulfan-based reduced-intensity conditioning and HLA-matched HSCT cure hemophagocytic lymphohistiocytosis

  • Article/chapterEnglish2020

Publisher, publication year, extent ...

  • 2020-05-08
  • American Society of Hematology,2020

Numbers

  • LIBRIS-ID:oai:gup.ub.gu.se/294250
  • https://gup.ub.gu.se/publication/294250URI
  • https://doi.org/10.1182/bloodadvances.2020001748DOI
  • http://kipublications.ki.se/Default.aspx?queryparsed=id:143685111URI

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  • Language:English

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  • Subject category:ref swepub-contenttype
  • Subject category:art swepub-publicationtype

Notes

  • Reduced-intensity/reduced-toxicity conditioning and allogeneic T-cell replete hematopoietic stem cell transplantation are curative in patients with hemophagocytic lymphohistiocytosis (HLH). Unstable donor chimerism (DC) and relapses are clinical challenges. We examined the effect of a reduced-intensity conditioning regimen based on targeted busulfan to enhance myeloid DC in HLH. The European Society for Bone and Marrow Transplantation-approved reduced-intensity conditioning protocol comprised targeted submyeloablative IV busulfan, IV fludarabine, and serotherapy comprising IV alemtuzumab (0.5-0.8 mg/kg) for unrelated-donor and IV rabbit anti-T-cell globulin for related-donor transplants. We assessed toxicity, engraftment, graft-versus-host disease (GHVD), DC in blood cell subtypes, and overall survival/event-free survival. Twenty-five patients from 7 centers were treated (median age, 0.68 year). The median total dose and cumulative area under the curve of busulfan was 13.1 mg/kg (6.4-26.4) and 63.1 mg/L x h (48-77), respectively. Bone marrow, peripheral blood stem cell, or cord blood transplants from HLA-matched related (n = 7) or unrelated (n = 18) donors were administered. Donor cells engrafted in all patients (median: neutrophils d+20/platelets d128). At last follow-up (median, 36 months; range, 8-111 months), the median DC of CD151 neutrophils, CD3(+) T cells, and CD16(+)56(+) natural killer cells was 99.5% (10-100), 97% (30-100), and 97.5% (30-100), respectively. Eight patients (32%) developed sinusoidal obstruction syndrome, resolving after defibrotide treatment. The 3-year overall survival and event-free survival rates were both 100%. None of the patients developed acute grade III to IV GHVD. Limited chronic GVHD was encountered in 4%. This regimen achieves excellent results with stable DC in patients with HLH.

Subject headings and genre

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  • Steward, C. G. (author)
  • Kentouche, K. (author)
  • Fasth, Anders,1945Gothenburg University,Göteborgs universitet,Institutionen för kliniska vetenskaper, Avdelningen för pediatrik,Institute of Clinical Sciences, Department of Pediatrics(Swepub:gu)xfasan (author)
  • Wynn, R. F. (author)
  • Zeilhofer, U. (author)
  • Haunerdinger, V. (author)
  • Volkmer, B. (author)
  • Prader, S. (author)
  • Gruhn, B. (author)
  • Ehl, S. (author)
  • Lehmberg, K. (author)
  • Muller, D. (author)
  • Gennery, A. R. (author)
  • Albert, M. H.Karolinska Institutet (author)
  • Hauck, F. (author)
  • Rao, K. (author)
  • Veys, P. (author)
  • Hassan, M. (author)
  • Lankester, A. C. (author)
  • Schmid, J. P. (author)
  • Hauri-Hohl, M. M. (author)
  • Gungor, T. (author)
  • Inborn Errors Working Party, Iewp E. (author)
  • European Soc Immunodeficiencies, E. S. (author)
  • Göteborgs universitetInstitutionen för kliniska vetenskaper, Avdelningen för pediatrik (creator_code:org_t)

Related titles

  • In:Blood Advances: American Society of Hematology4:9, s. 1998-20102473-95292473-9537

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