Humoral Primary Immunodeficiencies

Mario M. D'Elios, MD, PhD, Specialist in Clinical Immunology & Allergy, Professor of Internal Medicine, Group leader, chief of the Nanomedicine and Immunology unit at the University of Florence, Italy. Expert in mucosal immunology, primary immunodeficiencies and infectious diseases.Marta Rizzi, MD, PhD, Specialist in Allergy and Clinical Immunology, Group leader, Margarete von Wrangell fellow, at the Department of Rheumatology and Clinical Immunology /Centre of Chronic Immunodeficiency, University Medical Centre Freiburg, Germany, Expert in human B lymphocytes development, primary immunodeficiency and immune dysregulation.

• Preface.- 1. B cells and the B-cell immunological synapse.-2  Mucosal B cells.- 3 CVID. – 4 LOCID. – 5 Genetics of CVID. – 6 ICOS deficiency. – 7 CD19 deficiency.- 8 CD20 deficiency. – 9 CD21 deficiency. – 10 CD81 deficiency. - 11 TACI deficiency, . – 12 LRBA deficiency. – 13 BAFF receptor deficiency. – 14 TWEAK defects. - 15 NFKB2 deficiency. – 16 WHIM syndrome. – 17 Novel humoral PID genes. – 18 Class Switch Recombination defects,.- 19 Selective IgA deficiency.- 20 IgA with IgG subclass deficiency. - 21 Isolated IgG subclass deficiency. – 22 Thymoma with immunodeficiency. – 23 PRKC delta and PI3K-delta associated diseases. – 24 Differential diagnostic in hypogammaglobulinemia. – 25 Management of Humoral primary immunodeficiencies in pediatrics.- 26 Management of Humoral primary immunodeficiencies in adults.- 27 Vaccines in humoral PID.- 28 Humoral PID and neoplasias.- 29 Humoral PID and autoimmunity. – 30 Lung involvement.- 31 Gut involvement.- Subject Index.