B-cell differentiation and IL-21 response in IL2RG/JAK3 SCID patients after hematopoietic stem cell transplantation

Author:

Miggelbrink Alexandra M.12,Logan Brent R.3,Buckley Rebecca H.45,Parrott Roberta E.4,Dvorak Christopher C.6,Kapoor Neena78,Abdel-Azim Hisham78,Prockop Susan E.9,Shyr David10,Decaluwe Hélène11,Hanson Imelda C.12,Gillio Alfred13,Dávila Saldaña Blachy J.14,Eibel Hermann1516,Hopkins Gregory12,Walter Jolan E.17,Whangbo Jennifer S.12,Kohn Donald B.18,Puck Jennifer M.6,Cowan Morton J.6,Griffith Linda M.19,Haddad Elie1120,O’Reilly Richard J.9,Notarangelo Luigi D.21,Pai Sung-Yun12ORCID

Affiliation:

1. Division of Pediatric Hematology-Oncology, Boston Children’s Hospital, Boston, MA;

2. Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA;

3. Division of Biostatistics, Medical College of Wisconsin, Milwaukee, WI;

4. Department of Pediatrics and

5. Department of Immunology, Duke University Medical Center, Durham, NC;

6. Division of Allergy/Immunology/Blood & Marrow Transplantation, Benioff Children’s Hospital, University of California, San Francisco, San Francisco, CA;

7. Division of Hematology, Oncology, and Blood and Marrow Transplantation, Children’s Hospital Los Angeles, Los Angeles, CA;

8. University of Southern California Keck School of Medicine, Los Angeles, CA;

9. Bone Marrow Transplant Program, Memorial Sloan Kettering Cancer Center, New York, NY;

10. Division of Hematology, Primary Children’s Hospital, University of Utah/Huntsman Cancer Institute, Salt Lake City, UT;

11. Department of Pediatrics, University of Montreal, CHU Sainte-Justine, Montreal, QC, Canada;

12. Section of Pediatric Immunology, Allergy and Rheumatology, Baylor College of Medicine, Texas Children’s Hospital, Houston, TX;

13. Pediatric Blood and Marrow Transplant Program, Hackensack University Medical Center, Hackensack, NJ;

14. Division of Blood and Marrow Transplantation & Allergy and Immunology, Children’s National Medical Center, Washington, DC;

15. Centre of Chronic Immunodeficiency, Medical Center, and

16. Faculty of Medicine, University of Freiburg, Freiburg, Germany;

17. Division of Pediatric Allergy and Immunology, Massachusetts General Hospital for Children, Boston, MA;

18. Mattel Children’s Hospital, University of California, Los Angeles, Mattel Children’s Hospital, Los Angeles, CA;

19. Division of Allergy, Immunology and Transplantation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD;

20. Department of Microbiology, Infectious Disease and Immunology, University of Montreal, CHU Sainte-Justine, Montreal, QC, Canada; and

21. Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD

Abstract

Key Points IL2RG/JAK3-deficient B cells remain intrinsically defective posttransplant despite follicular helper T-cell reconstitution. In vitro response of B cells to IL-21 is a potential biomarker for humoral immunity in patients with IL2RG/JAK3 SCID after transplantation.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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