Hartz, Bernd and Marsh, Rebecca and Rao, Kanchan and Henter, Jan-Inge and Jordan, Michael and Filipovich, Lisa and Bader, Peter and Beier, Rita and Burkhardt, Birgit and Meisel, Roland and Schulz, Ansgar and Winkler, Beate and Albert, Michael H. and Greil, Johann and Karasu, Gulsun and Woessmann, Wilhelm and Corbacioglu, Selim and Gruhn, Bernd and Holter, Wolfgang and Kuehl, Joern-Sven and Lang, Peter and Seidel, Markus G. and Veys, Paul and Lofstedt, Alexandra and Ammann, Sandra and Ehl, Stephan and Janka, Gritta and Mueller, Ingo and Lehmberg, Kai (2016) The minimum required level of donor chimerism in hereditary hemophagocytic lymphohistiocytosis. BLOOD, 127 (25). pp. 3281-3290. ISSN 0006-4971, 1528-0020
Full text not available from this repository. (Request a copy)Abstract
Reduced-intensity conditioning has improved survival after hematopoietic stem cell transplantation (HSCT) for hemophagocytic lymphohistiocytosis (HLH) at the cost of more frequent mixed chimerism. The minimum level of donor chimerism (DC) required to prevent HLH reactivation in humans remains to be determined. In a multicenter retrospective study, 103 patients transplanted for hereditary HLH (2000-2013) and DC permanently or transiently <75% (overall, CD3(+), CD56(+)) were analyzed regarding DC, specific immunologic function, occurrence of systemic reactivations (>= 5/8 HLH criteria), partial systemic flares (< 5 criteria and HLH-directed treatment), isolated central nervous system reactivations, and management. Recurrence was reported in 18 patients (systemic reactivation n = 11, partial flare n = 3, isolated central nervous system reactivation n = 4). Tenevents occurred during profound immune suppression before day 180 (median DC, 10%; range, 1-100%; CD3(+) if available, otherwise overall DC), which renders a differentiation between secondary post-HSCT HLH and HLH related to the genetic defect difficult. Eight events occurred between 0.5 and 6.7 years post-HSCT (median DC, 13%; range, 0-30%). In 5 patients, overall and lineage-specific DC were <= 10% for >6 months (median, 5.1; range, 1.1-10 years) without reactivation. A second HSCT was performed in 18 patients (median, DC 4%; range, 0-19%). Death from reactivation occurred in 4 patients (22% of recurrences). Six patients died of transplant complications following a second HSCT(33% of second HSCT). We conclude that a DC>20%-30% is protective against late reactivation. Lower levels do not, however, inescapably result in recurrences. The decision for or against second HSCT must be based on a thorough risk assessment.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | STEM-CELL TRANSPLANTATION; XIAP DEFICIENCY; HISTIOCYTOSIS; ALEMTUZUMAB; MUTATIONS; ETOPOSIDE; DIAGNOSIS; DISTINCT; OUTCOMES; DISEASE; |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Abteilung für Pädiatrische Hämatologie, Onkologie und Stammzelltransplantation |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 08 Apr 2019 09:02 |
| Last Modified: | 08 Apr 2019 09:02 |
| URI: | https://pred.uni-regensburg.de/id/eprint/3752 |
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