Antibody Deficiency PDF
Preview Antibody Deficiency
ACAAI/AAAAI Joint Board Certification Review Course Syllabus Nashville 2018 PROVIDED by the American College of Allergy, Asthma and Immunology in cooperation with the American Academy of Allergy, Asthma & Immunology. © 2018 American College of Allergy, Asthma & Immunology. Distribution is prohibited. Table of Contents Friday, May 18, 2018 T cell Biology 1 Troy Torgerson, MD, PhD B cell Biology and Immunoglobulin 19 Structure James “Tom” Thomas, II, MD Innate Immunity (NK cells, Pattern Recognition Receptors) 42 Jordan Orange, MD, PhD Innate Immune Disorders (NK defects, GATA2, XLP, HLH, TLR defects) 67 Jordan Orange, MD, PhD Cell Signaling 102 John Routes, MD Autoinflammatory Syndromes 130 Karyl Barron, MD Laboratory Testing 151 Thomas Fleisher, MD Cytokines 174 Robert Schleimer, PhD Genetics and Molecular Biology 192 Sergio Rosenzweig, MD, PhD T cell Immunodeficiency 210 Thomas Fleisher, MD B cell Immunodeficiency 230 John Routes, MD Saturday, May 19, 2018 Disorders of Phagocytes and Macrophages 254 Steven Holland, MD Mast Cell and Basophil Biology and Disorders 309 Matthew Giannetti, MD Eosinophil Biology and Disorders 335 Paneez Khoury, MD, MHSc IV/SC Ig and Biologic Modifiers 359 Mark Ballow, MD HLA and Transplantation 382 Troy Torgerson, MD, PhD Atopic Dermatitis 395 Kelly Stone, MD, PhD Urticaria and Angioedema 419 David Khan, MD Anaphylaxis 457 David Lang, MD Systemic Autoimmunity 488 Carol Langford, MD, MHS Allergen Immunotherapy 514 Mike Tankersley, MD, MBA Vasculitis 541 Carol Langford, MD, MHS Drug Allergy 571 David Khan, MD Sunday, May 20, 2018 Pharmacology 630 Joseph Spahn, MD Asthma (and VCD): Pathophysiology, Diagnosis, Treatment 677 David Lang, MD Food Allergy 713 Carla Davis, MD Bullous Dermatoses, Immunologic Skin Disease, Contact Dermatitis 761 Anthony Gaspari, MD Occupational Asthma and Immunologic Lung Disease 798 (Hypersensitivity, Alpha 1-antitrypsin Deficiency, Cystic Fibrosis) Mark Sands, MD Rhinitis, Polyposis, Sinusitis and Otitis Media 860 Anju Peters, MD Review Questions Friday Section Review Questions 934 Friday Section Answers 947 Saturday Section Review Questions 952 Saturday Section Answers 962 Sunday Section Review Questions 970 Sunday Section Answers 974 T cell Biology Troy R. Torgerson, MD PhD Pediatrics, Immunology/Rheumatology University of Washington and Seattle Children’s Hospital Disclosures 1. Consulting: • Shire • CSL Behring • ADMA Biosciences • UCB Biopharmaceuticals 2. Research Funding: • National Institutes of Health • Shire • CSL Behring • Casebia Therapeutics 2018 ACAAI/AAAAI Joint Board Certification Review Course 1 Learning Objectives 1. Describe the general structure & composition of the T cell Receptor Complex 2. Describe how T cells get activated through the TCR 3. Describe the general mechanisms involved in thymic development of T cells 4. Describe the functions of different T cell subsets 5. Describe the clinical consequences of T cell deficiency. The T cell Receptor Complex TCR Recognition Ag Binding CD3 CD3 Site V V a b C C a b ITAMs Signaling 2018 ACAAI/AAAAI Joint Board Certification Review Course 2 Process of Recombination • Similar to B cell VDJ recombination • Recombination of V, D, and J gene segments is coordinated by recombinase-activating genes RAG-1 and RAG-2 (like in B cells) and repair done by double- strand break repair proteins. • Unlike Ig genes, TCR genes do not undergo class switching or somatic mutation. There is no change in TCR structure or affinity during activation, differentiation, and expansion of T cells. Once a TCR is set, it is set. Learning Objectives 1. Describe the general structure & composition of the T cell Receptor Complex 2. Describe how T cells get activated through the TCR 3. Describe the general mechanisms involved in thymic development of T cells 4. Describe the functions of different T cell subsets 5. Describe the clinical consequences of T cell deficiency. 2018 ACAAI/AAAAI Joint Board Certification Review Course 3 T cell Activation via TCR – MHC Interaction • T cell activation depends on both the strength and duration of TCR signaling • Full T cell activation requires prolonged T cell- APC interactions • T cell-APC interactions need to be stabilized to be effective. • Adhesion molecules provide this stabilization T cell Activation Requires Multiple Signals 2018 ACAAI/AAAAI Joint Board Certification Review Course 4 • Initial binding of T cell APC with APC is through T cell adhesion molecules MHC II like LFA-1 ICAM • If no TCR activation, T CD4 cells let go and move on. • If TCR is activated, this V V CD3 a b activates the integrins to LFA-1 C C a high affinity state and a b recruits additional integrins to the point of cell-cell contact. T cell TCR The Immunologic Synapse BasuR and HuseM (2017) Trends in Cell Biology 27:241-54 • Requires the actin cytoskeleton to organize the synapse. • Problems with organizing/assembling actin (WAS deficiency) lead to poor synapse formation. 2018 ACAAI/AAAAI Joint Board Certification Review Course 5
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