Immune reconstitution and survival of 100 SCID patients post–hematopoietic cell transplant: a PIDTC natural history study

Author:

Heimall Jennifer1,Logan Brent R.2,Cowan Morton J.3,Notarangelo Luigi D.4,Griffith Linda M.5,Puck Jennifer M.3,Kohn Donald B.6,Pulsipher Michael A.7,Parikh Suhag8,Martinez Caridad9,Kapoor Neena7,O’Reilly Richard10,Boyer Michael11,Pai Sung-Yun12,Goldman Frederick13,Burroughs Lauri14,Chandra Sharat15,Kletzel Morris16,Thakar Monica17,Connelly James18,Cuvelier Geoff19,Davila Saldana Blachy J.20,Shereck Evan21,Knutsen Alan22,Sullivan Kathleen E.1,DeSantes Kenneth23,Gillio Alfred24,Haddad Elie25,Petrovic Aleksandra26,Quigg Troy27,Smith Angela R.28,Stenger Elizabeth29,Yin Ziyan2,Shearer William T.9,Fleisher Thomas30,Buckley Rebecca H.8,Dvorak Christopher C.3ORCID

Affiliation:

1. Division of Allergy and Immunology, Children’s Hospital of Philadelphia, Philadelphia, PA;

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

3. Division of Pediatric Allergy, Immunology and Bone Marrow Transplant, University of California, San Francisco, San Francisco, CA;

4. Laboratory of Host Defense, Division of Intramural Research, and

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

6. Department of Pediatrics, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA;

7. Children's Hospital Los Angeles, University of Southern California, Los Angeles, CA;

8. Department of Pediatrics, Duke University, Durham, NC;

9. Texas Children's Hospital, Baylor College of Medicine, Houston, TX;

10. Memorial Sloan Kettering Cancer Center, New York, NY;

11. University of Utah Health, Salt Lake City, UT;

12. Boston Children's Hospital, Boston, MA;

13. Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL;

14. Fred Hutchinson Cancer Research Center, Seattle, WA;

15. Cincinnati Children's Hospital, Cincinnati, OH;

16. Division of Pediatric Hematology, Oncology and Stem Cell Transplantation, Northwestern University, Evanston, IL;

17. Division of Pediatric Hematology/Oncology/Transplant, Medical College of Wisconsin, Milwaukee, WI;

18. Department of Pediatrics, Vanderbilt University, Nashville, TN;

19. CancerCare Manitoba, Winnipeg, MB, Canada;

20. Division of Blood and Marrow Transplantation, Children's National Medical Center, Washington, DC;

21. Division of Pediatric Hematology/Oncology, Department of Pediatrics, Oregon Health and Science University, Portland, OR;

22. Cardinal Glennon Children's Hospital, St. Louis, MO;

23. University of Wisconsin–Madison, Madison, WI;

24. Hackensack University Medical Center, Hackensack, NJ;

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

26. Seattle Children's Hospital, Seattle, WA;

27. Methodist Children's Hospital of South Texas, San Antonio, TX;

28. Division of Blood and Marrow Transplantation, Department of Pediatrics, University of Minnesota, Minneapolis, MN;

29. Children's Healthcare of Atlanta, Atlanta, GA; and

30. Clinical Center, National Institutes of Health, Bethesda, MD

Abstract

Key Points Active infection pretransplant adversely impacts survival (81% in patients with active infection vs 95% in infection-free patients; P = .009). Preparative chemotherapy improved 1-year post-HCT median CD4 counts (P = .02) and freedom from IV immunoglobulin (P < .001).

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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