Springer, 2019. — 383 p. — (Rare Diseases of the Immune System). — ISBN: 3319917846.
This book presents detailed state of the art knowledge on the humoral primary immunodeficiencies (PIDs), i.e., disorders arising from impaired antibody production due to defects intrinsic to B cells or defective interaction between B and T cells. There is extensive coverage of both basic science discoveries and the latest clinical advances in the field. The book is structured in accordance with the most recent classification of PIDs and also covers updates on the B cell immunological synapse. Readers will find comprehensive, in-depth descriptions of novel humoral PID genes and related clinical applications, mucosal B cells, and the various clinical phenotypes of humoral PIDs. Aspects such as differential diagnosis, clinical management in children and adults, and the role of vaccines are also addressed.
The B-Side of the Immune Response
Mucosal B Cells
CVID
Late-Onset Combined Immunodeficiencies (LOCID)
Genetics of CVID
ICOS Deficiency
CD19 Deficiency due to Genetic Defects in the CD19 and CD81 Genes
Genetic CD21 Deficiency
TACI Deficiency
LRBA Deficiency
BAFF Receptor Deficiency
TWEAK Deficiency
NFKB2 Defects
The WHIM Syndrome
Class-Switch Recombination Defects
Selective IgA Deficiency
IgG Subclass and Anti-polysaccharide Antibody Deficiency
Good’s Syndrome (GS): Thymoma with Immunodeficiency
Differential Diagnosis in Hypogammaglobulinemia
Management of Humoral Primary Immunodeficiencies in Pediatrics
Management of Humoral Primary Immunodeficiencies in Adults
Vaccines in Humoral Primary Immunodeficiencies
Malignancy in Predominantly Antibody Deficiencies (PAD)
Humoral Primary Immunodeficiency and Autoimmune and Inflammatory Manifestations
Chest Complications in Patients with Primary Antibody Deficiency Syndromes (PADS)
Gastrointestinal Complications in Primary Immunoglobulin Deficiencies