Diagnostic assay to assist clinical decisions for unclassified severe combined immune deficiency

Blood Adv. 2020 Jun 23;4(12):2606-2610. doi: 10.1182/bloodadvances.2020001736.

Panojot Bifsha¹, Jennifer W Leiding², Sung-Yun Pai^{3

4}, Aurelien B L Colamartino^{1

5}, Nicholas Hartog^{6

7}, Joseph A Church⁸, Benjamin R Oshrine⁹, Jennifer M Puck^{10

11}, M Louise Markert^{12

13}, Elie Haddad^{1

5

14}

¹ CHU Sainte-Justine Research Center, Montreal, QC, Canada.
² Division of Allergy and Immunology, Department of Pediatrics, University of South Florida at Johns Hopkins-All Children's Hospital, St. Petersburg, FL.
³ Division of Hematology-Oncology, Boston Children's Hospital, Boston, MA.
⁴ Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA.
⁵ Department of Microbiology, Infectious Diseases and Immunology, University of Montreal, Montreal, QC, Canada.
⁶ Department of Allergy and Immunology, Spectrum Health/Helen DeVos Children's Hospital, Grand Rapids, MI.
⁷ Michigan State College of Human Medicine, Grand Rapids, MI.
⁸ Clinical Immunology and Allergy, Children's Hospital Los Angeles and Department of Pediatrics, Keck School of Medicine of the University of Southern California, Los Angeles, CA.
⁹ Cancer and Blood Disorders Institute, Johns Hopkins-All Children's Hospital, St. Petersburg, FL.
¹⁰ Division of Allergy, Immunology and Blood and Marrow Transplantation, Department of Pediatrics, University of California, San Francisco, San Francisco, CA.
¹¹ UCSF Benioff Children's Hospital, San Francisco, CA.
¹² Department of Pediatrics and.
¹³ Department of Immunology, Duke University Medical Center, Durham, NC; and.
¹⁴ Department of Pediatrics, University of Montreal, Montreal, QC, Canada.

Abstract

3D organoid T-cell differentiation from a few hundred peripheral blood CD34⁺ cells was successfully achieved.
3D organoid T-cell differentiation could help physicians distinguish intrinsic from extrinsic defects underlying a clinical SCID phenotype.