Overview of Immunodeficiency Disorders

Abstract

The spectrum of primary immunodeficiency disorders (PIDs) is expanding. It includes typical disorders that primarily present with defective immunity as well as disorders that predominantly involve other systems and show few features of impaired immunity. The rapidly growing list of new immunodeficiency disorders and treatment modalities makes it imperative for providers to stay abreast of the latest and best management strategies. This article presents a brief overview of recent clinical advances in PIDs.

Keywords: Antibody deficiency; Autoimmunity; Immune defect; Immune dysregulation; Immunodeficiency; Innate immune defect; Lymphoproliferation.

Figure 1

Features of Primary Immunodeficiencies Data from Refs ^{, , -, -}

Figure 2

Diagnostic tests in evaluation of Primary Immunodeficiencies Data from Locke BA, Dasu T, Verbsky JW. Laboratory diagnosis of primary immunodeficiencies. Clinical reviews in allergy & immunology. Apr 2014;46(2):154-168. A. Basic Immunology work up B. Advanced immunology work up based on clinical and laboratory clues TRECs, T cell receptor excision circles; NK, natural killer; TLR, toll-like receptor; ALPS, autoimmune lymphoproliferative syndrome, IgD, immunoglobulin D. C. Molecular testing

Figure 2

Diagnostic tests in evaluation of Primary Immunodeficiencies Data from Locke BA, Dasu T, Verbsky JW. Laboratory diagnosis of primary immunodeficiencies. Clinical reviews in allergy & immunology. Apr 2014;46(2):154-168. A. Basic Immunology work up B. Advanced immunology work up based on clinical and laboratory clues TRECs, T cell receptor excision circles; NK, natural killer; TLR, toll-like receptor; ALPS, autoimmune lymphoproliferative syndrome, IgD, immunoglobulin D. C. Molecular testing

Figure 2

Diagnostic tests in evaluation of Primary Immunodeficiencies Data from Locke BA, Dasu T, Verbsky JW. Laboratory diagnosis of primary immunodeficiencies. Clinical reviews in allergy & immunology. Apr 2014;46(2):154-168. A. Basic Immunology work up B. Advanced immunology work up based on clinical and laboratory clues TRECs, T cell receptor excision circles; NK, natural killer; TLR, toll-like receptor; ALPS, autoimmune lymphoproliferative syndrome, IgD, immunoglobulin D. C. Molecular testing

Figure 3

Therapies for various types of Primary Immunodeficiencies Ig, immunoglobulin; G-CSF, granulocyte-colony-stimulating factor Data from Refs ^{, , -}