The minimum required level of donor chimerism in hereditary hemophagocytic lymphohistiocytosis

Author:

Hartz Bernd1ORCID,Marsh Rebecca2,Rao Kanchan3,Henter Jan-Inge4,Jordan Michael2,Filipovich Lisa2,Bader Peter5,Beier Rita67,Burkhardt Birgit8,Meisel Roland9,Schulz Ansgar10,Winkler Beate111,Albert Michael H.12,Greil Johann13,Karasu Gülsün14,Woessmann Wilhelm15,Corbacioglu Selim16,Gruhn Bernd17,Holter Wolfgang1819,Kühl Jörn-Sven20,Lang Peter21,Seidel Markus G.1822,Veys Paul3,Löfstedt Alexandra423,Ammann Sandra24,Ehl Stephan24,Janka Gritta1,Müller Ingo1,Lehmberg Kai1ORCID

Affiliation:

1. Department of Pediatric Hematology and Oncology, University Medical Center Hamburg Eppendorf, Hamburg, Germany;

2. Cincinnati Children’s Hospital, Cincinnati, OH;

3. Great Ormond Street Hospital, London, United Kingdom;

4. Childhood Cancer Research Unit, Department of Women’s and Children’s Health, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden;

5. Division for Stem Cell Transplantation and Immunology, University Children’s Hospital, Frankfurt am Main, Germany;

6. Department of Pediatric Hematology and Oncology, Hannover Medical School, Hannover, Germany;

7. Department of Pediatric Hematology and Oncology, University Medical Center Essen, Germany;

8. Department of Pediatric Hematology and Oncology, University Medical Center Münster, Münster, Germany;

9. Department of Pediatric Hematology, Oncology and Immunology, University Children's Hospital Düsseldorf, Düsseldorf, Germany;

10. Department of Pediatric and Adolescent Medicine, University Hospital Ulm, Ulm, Germany;

11. Department of Pediatric Hematology/Oncology and Stem Cell Transplantation, University Children’s Hospital, Würzburg, Germany;

12. Dr. von Hauner University Children’s Hospital, Ludwig-Maximilian University Munich, Munich, Germany,

13. Department of Pediatric Hematology and Oncology, University Children’s Hospital Heidelberg, Heidelberg, Germany;

14. Pediatric Stem Cell Transplantation Unit, Goztepe Medicalpark Hospital, Bahcesehir University, Istanbul, Turkey;

15. Department of Pediatric Hematology and Oncology, University Children’s Hospital, Giessen, Germany;

16. Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, University Children’s Hospital, Regensburg, Germany;

17. Department of Pediatrics, Jena University Hospital, Jena, Germany;

18. St. Anna Children’s Hospital, Medical University Vienna, Vienna, Austria;

19. Department of Pediatric Hematology and Oncology, University Medical Center Erlangen, Erlangen, Germany;

20. Department of Pediatric Oncology, Hematology, and Stem Cell Transplantation, Charité, Berlin, Germany;

21. Department of Hematology and Oncology, University Children's Hospital Tübingen, Tübingen, Germany;

22. Division of Pediatric Hematology-Oncology, Department of Pediatrics and Adolescent Medicine, Medical University Graz, Graz, Austria;

23. Department of Clinical Science, Intervention and Technology, Karolinska Institutet, and Hematology/Immunology Section, Astrid Lindgren Children’s Hospital, Karolinska University Hospital Huddinge, Stockholm, Sweden; and

24. Center for Chronic Immunodeficiency, University Medical Center Freiburg, Freiburg, Germany

Abstract

Key Points Donor chimerism >20%-30% usually protects against late disease reactivation after day 180 post stem cell transplantation for primary HLH. Lower levels do not inevitably result in reactivations. The risks of intervention must be weighed against the risk of reactivation.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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