Artemio M Jongco1,2,3,4, Robert Sporter5,6, Elise Hon7, Omer Elshaigi7, Shouling Zhang8,6, Foysal Daian7, Emily Bae7, Amanda Innamorato7, Catherine Capo7, Brianne Navetta-Modrov9, David W Rosenthal7,8,10, Vincent R Bonagura7,8,10,11. 1. Division of Allergy & Immunology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, USA. ajongco@northwell.edu. 2. Department of Pediatrics, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, USA. ajongco@northwell.edu. 3. Department of Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, USA. ajongco@northwell.edu. 4. Center for Health Innovations and Outcomes Research, Feinstein Institutes for Medical Research, Manhasset, NY, USA. ajongco@northwell.edu. 5. ENT and Allergy Associates, New York, NY, USA. 6. Division of Allergy & Clinical Immunology, Icahn School of Medicine at Mount Sinai, New York, NY, USA. 7. Division of Allergy & Immunology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, USA. 8. Department of Pediatrics, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, USA. 9. Division of Rheumatology, Allergy & Immunology, Stony Brook University School of Medicine, East Setauket, NY, USA. 10. Department of Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, USA. 11. Center for Molecular Medicine, Feinstein Institutes for Medical Research, Manhasset, NY, USA.
Abstract
PURPOSE: Newborn screening (NBS) quantifies T cell receptor excision circles (TREC) and identifies infants with T cell lymphopenia (TCL). This study elucidates the demographics, laboratory characteristics, genetics, and clinical outcomes following live viral vaccine administration of term infants with transient or persistent idiopathic TCL. METHODS: A single-center retrospective analysis was performed from September 2010 through June 2018. Laboratory variables were compared with Mann-Whitney tests. Correlations between initial TREC levels and T cell counts were determined by Spearman tests. RESULTS: Twenty-two transient and 21 persistent TCL infants were identified. Males comprised 68% of the transient and 52% of the persistent TCL cohorts. Whites comprised 23% of the transient and 29% of the persistent cohorts. Median initial TREC levels did not differ (66 vs. 60 TRECs/μL of blood, P = 0.58). The transient cohort had higher median initial CD3+ (2135 vs. 1169 cells/μL, P < 0.001), CD4+ (1460 vs. 866 cells/μL, P < 0.001), and CD8+ (538 vs. 277 cells/μL, P < 0.001) counts. The median age of resolution for the transient cohort was 38 days. Genetic testing revealed 2 genes of interest which warrant further study and several variants of uncertain significance in immunology-related genes in the persistent cohort. 19 transient and 14 persistent subjects received the initial rotavirus and/or MMRV immunization. No adverse reactions to live viral vaccines were reported in either cohort. CONCLUSION: Transient and persistent TCL infants differ by demographic, laboratory, and clinical characteristics. Select transient and persistent TCL patients may safely receive live attenuated viral vaccines, but larger confirmatory studies are needed.
PURPOSE: Newborn screening (NBS) quantifies T cell receptor excision circles (TREC) and identifies infants with T cell lymphopenia (TCL). This study elucidates the demographics, laboratory characteristics, genetics, and clinical outcomes following live viral vaccine administration of term infants with transient or persistent idiopathic TCL. METHODS: A single-center retrospective analysis was performed from September 2010 through June 2018. Laboratory variables were compared with Mann-Whitney tests. Correlations between initial TREC levels and T cell counts were determined by Spearman tests. RESULTS: Twenty-two transient and 21 persistent TCL infants were identified. Males comprised 68% of the transient and 52% of the persistent TCL cohorts. Whites comprised 23% of the transient and 29% of the persistent cohorts. Median initial TREC levels did not differ (66 vs. 60 TRECs/μL of blood, P = 0.58). The transient cohort had higher median initial CD3+ (2135 vs. 1169 cells/μL, P < 0.001), CD4+ (1460 vs. 866 cells/μL, P < 0.001), and CD8+ (538 vs. 277 cells/μL, P < 0.001) counts. The median age of resolution for the transient cohort was 38 days. Genetic testing revealed 2 genes of interest which warrant further study and several variants of uncertain significance in immunology-related genes in the persistent cohort. 19 transient and 14 persistent subjects received the initial rotavirus and/or MMRV immunization. No adverse reactions to live viral vaccines were reported in either cohort. CONCLUSION: Transient and persistent TCL infants differ by demographic, laboratory, and clinical characteristics. Select transient and persistent TCL patients may safely receive live attenuated viral vaccines, but larger confirmatory studies are needed.
Entities:
Keywords:
Idiopathic lymphopenia; New York State; T cell receptor excision circle assay; newborn screening
Authors: Jacalyn L Gerstel-Thompson; Jonathan F Wilkey; Jennifer C Baptiste; Jennifer S Navas; Sung-Yun Pai; Kenneth A Pass; Roger B Eaton; Anne Marie Comeau Journal: Clin Chem Date: 2010-07-21 Impact factor: 8.327
Authors: James W Verbsky; Mei W Baker; William J Grossman; Mary Hintermeyer; Trivikram Dasu; Benedetta Bonacci; Sreelatha Reddy; David Margolis; James Casper; Miranda Gries; Ken Desantes; Gary L Hoffman; Charles D Brokopp; Christine M Seroogy; John M Routes Journal: J Clin Immunol Date: 2011-11-10 Impact factor: 8.317
Authors: John M Routes; William J Grossman; James Verbsky; Ronald H Laessig; Gary L Hoffman; Charles D Brokopp; Mei W Baker Journal: JAMA Date: 2009-12-09 Impact factor: 56.272
Authors: Antonia Kwan; Roshini S Abraham; Robert Currier; Amy Brower; Karen Andruszewski; Jordan K Abbott; Mei Baker; Mark Ballow; Louis E Bartoshesky; Francisco A Bonilla; Charles Brokopp; Edward Brooks; Michele Caggana; Jocelyn Celestin; Joseph A Church; Anne Marie Comeau; James A Connelly; Morton J Cowan; Charlotte Cunningham-Rundles; Trivikram Dasu; Nina Dave; Maria T De La Morena; Ulrich Duffner; Chin-To Fong; Lisa Forbes; Debra Freedenberg; Erwin W Gelfand; Jaime E Hale; I Celine Hanson; Beverly N Hay; Diana Hu; Anthony Infante; Daisy Johnson; Neena Kapoor; Denise M Kay; Donald B Kohn; Rachel Lee; Heather Lehman; Zhili Lin; Fred Lorey; Aly Abdel-Mageed; Adrienne Manning; Sean McGhee; Theodore B Moore; Stanley J Naides; Luigi D Notarangelo; Jordan S Orange; Sung-Yun Pai; Matthew Porteus; Ray Rodriguez; Neil Romberg; John Routes; Mary Ruehle; Arye Rubenstein; Carlos A Saavedra-Matiz; Ginger Scott; Patricia M Scott; Elizabeth Secord; Christine Seroogy; William T Shearer; Subhadra Siegel; Stacy K Silvers; E Richard Stiehm; Robert W Sugerman; John L Sullivan; Susan Tanksley; Millard L Tierce; James Verbsky; Beth Vogel; Rosalyn Walker; Kelly Walkovich; Jolan E Walter; Richard L Wasserman; Michael S Watson; Geoffrey A Weinberg; Leonard B Weiner; Heather Wood; Anne B Yates; Jennifer M Puck; Vincent R Bonagura Journal: JAMA Date: 2014-08-20 Impact factor: 56.272
Authors: Anne Marie Comeau; Jaime E Hale; Sung-Yun Pai; Francisco A Bonilla; Luigi D Notarangelo; Mark S Pasternack; H Cody Meissner; Ellen Rae Cooper; Alfred DeMaria; Inderneel Sahai; Roger B Eaton Journal: J Inherit Metab Dis Date: 2010-05-20 Impact factor: 4.982
Authors: Beth H Vogel; Vincent Bonagura; Geoffrey A Weinberg; Mark Ballow; Jason Isabelle; Lisa DiAntonio; April Parker; Allison Young; Charlotte Cunningham-Rundles; Chin-To Fong; Jocelyn Celestin; Heather Lehman; Arye Rubinstein; Subhadra Siegel; Leonard Weiner; Carlos Saavedra-Matiz; Denise M Kay; Michele Caggana Journal: J Clin Immunol Date: 2014-03-01 Impact factor: 8.317