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Patients with common variable immunodeficiency with autoimmune cytopenias exhibit hyperplastic yet inefficient germinal center responses
Authors:Neil Romberg  Carole Le Coz  Salomé Glauzy  Jean-Nicolas Schickel  Melissa Trofa  Brian E. Nolan  Michele Paessler  Mina L. Xu  Michele P. Lambert  Saquib A. Lakhani  Mustafa K. Khokha  Soma Jyonouchi  Jennifer Heimall  Patricia Takach  Paul J. Maglione  Jason Catanzaro  F. Ida Hsu  Kathleen E. Sullivan  Eric Meffre
Affiliation:1. Division of Immunology and Allergy, Children''s Hospital of Philadelphia, Philadelphia, Pa;2. Division of Rheumatology, Children''s Hospital of Philadelphia, Philadelphia, Pa;3. Division of Hematopathology, Children''s Hospital of Philadelphia, Philadelphia, Pa;4. Division of Hematology, Children''s Hospital of Philadelphia, Philadelphia, Pa;5. Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pa;6. Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pa;7. Department of Medicine, Division of Allergy and Immunology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pa;8. Department of Immunobiology, Yale University School of Medicine, New Haven, Conn;9. Department of Pathology, Yale University School of Medicine, New Haven, Conn;10. Department of Pediatrics, Yale University School of Medicine, New Haven, Conn;11. Department of Genetics, Yale University School of Medicine, New Haven, Conn;12. Department of Medicine, Yale University School of Medicine, New Haven, Conn;13. Pediatric Genomics Discovery Program, Yale University School of Medicine, New Haven, Conn;14. Division of Clinical Immunology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY
Abstract:
Keywords:Common variable immunodeficiency  autoimmune cytopenias  germinal center responses  somatic hypermutation  B-cell tolerance  commensal bacteria  follicular helper T cell  regulatory T cell  AIC  Autoimmune cytopenia  AID  Activation-induced cytidine deaminase  APC  Allophycocyanin  AVY  Ala-Val-Tyr  CQ  Circularity quotient  CSR  Class-switch recombination  Circulating follicular helper T  CVID  Common variable immunodeficiency  GC  Germinal center  HD  Healthy donor  ITP  Immune thrombocytopenia  NHS  Asn-His-Ser  PD-1  Programmed cell death protein 1  PE  Phycoerythrin  PerCP  Peridinin-chlorophyll-protein complex  SHM  Somatic hypermutation  Follicular helper T  Treg  Regulatory T  Tresp  Responder T
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